Hematology and Oncology:Question 1 A 67-year-old man in previously good health is hospitalized because of a 2-day history of fever

and diminished consciousness. The patient responds inconsistently to verbal commands. His temperature is 39.5 °C (103.1 °F); he has tachycardia, and his blood pressure is 80/58 mm Hg. There is no bleeding. His hemoglobin is 12.1 g/dL, leukocyte count is 29,000/μL with 80% neutrophils, and platelet count is 20,000/μL. Which of the following studies should be obtained in this patient? A. Bone marrow aspiration and biopsy B. Factor VIII level C. Measurement of platelet-associated IgG D. Measurements of fibrin D-dimer and total fibrinogen E. Bleeding time Hematology and Oncology:Question 2 A 35-year-old woman has completed 6 months of anticoagulation therapy for pulmonary emboli. She had been using oral contraceptives for the past 10 years but stopped when the pulmonary emboli were detected. There is no family history of thrombosis. Two weeks after the discontinuation of warfarin, she undergoes coagulation studies. She is found to be heterozygous for the prothrom bin G2021 0A mutation. On the basis of her history and laboratory evaluation, what is the best recommendation for this patient? A. Resume oral anticoagulation therapy at a target INR of 2 to 3 B. Resume oral anticoagulation at a target INR of 1 .5 to 2 and advise her that it is safe to begin taking oral contraceptives again C. Advise the patient to take 81 mg of aspirin each day D. Prescribe a combination of aspirin and dipyridamole E. Advise the patient not to resume oral contraceptives and that additional therapy is not required for the thrombotic event Hematology and Oncology:Question 3 A 22-year-old man with sickle cell disease has four to six painful crises per year that require parenteral hydration and narcotics. Two weeks ago the patient developed an acute chest characterized by fever, dyspnea, and the radiographic appearance of pulmonary infiltrates. The patient’s condition rapidly responded to aggressive exchange transfusions and empiric broad-spectrum antibiotics; he has been asymptomatic for 1 week. Laboratory studies: Hemoglobin 10.4 g/dL Hematocrit 32% Leukocyte count 12,000/μL Platelet count 277,000/μL Hemoglobin electrophoresis shows the following results. Hemoglobin A (α2 β2) 62% (normal > 97.5%) Hemoglobin A2(α2 δ2) 2% (normal< 2.5%) Hemoglobin F (α2γ2) 1% (normal 0%) Hemoglobin S (α2 βS2) 35% (normal 0%) What is the best next step in this patient’s management? A. Long-term transfusion therapy B. Hydroxyurea therapy C. Consultation for therapeutic gene therapy D. Consultation for therapeutic bone marrow transplantation

E. Long-term oxygen therapy Hematology and Oncology:Question 4 A 24-year-old woman is evaluated in the emergency department because of fever, chills, sore throat, and difficulty talking. Two months ago she was diagnosed with schizophrenia, and clozapine therapy was initiated, at which time her complete blood count was normal. On physical examination, her tonsils are swollen and red bilaterally; she has submandibular lymphadenopathy and high fever. Her temperature is 40 °C (104 °F), pulse rate 140/min, and blood pressure is 90/50 mm Hg. Her hemoglobin is 12 g/dL, hematocrit is 36%, leukocyte count is 2000/μL, and platelet count is 180,000/μL. Throat and blood cultures are obtained; broad-spectrum intravenous antibiotic therapy is initiated and clozapine is discontinued. Which of the following is the most appropriate next step for this patient? A. Bone marrow aspiration and biopsy B. Initiation of subcutaneous granulocyte colony-stimulating factor daily C. Granulocyte transfusion D. Search for a suitable bone marrow donor E. Test for HIV

Hematology and Oncology:Question 5 A 46-year-old woman with alcoholism is evaluated because of a 4-day history of nausea and vomiting after a recent binge. She has diffuse epigastric pain. She has been hospitalized intermittently for years for medical complications related to alcohol abuse, including cirrhosis with ascites and bleeding esophageal varices. On physical examination, her temperature is 37.3 °C (99.1 °F), pulse rate is 68/min, and blood pressure is 135/70 mm Hg. She has jaundice and is icteric. Her lungs are clear to auscultation. The remainder of her physical examination is unremarkable. Laboratory studies: Hemoglobin 7.1 g/dL Leukocyte count 4500/μL Platelet count 115,000/μL Mean corpuscular volume 107 fL Reticulocyte count 5.4% Serum alkaline phosphatase 592 U/L Serum aspartate aminotransferase 198 U/L Serum alanine aminotransferase 166 U/L Serum total bilirubin 9.2 mg/dL Serum indirect bilirubin6.4 mg/dL Serum albumin 2.2 g/dL Serum folate 12 ng/mL Which of the following diagnoses best explains the findings? A. Spur cell anemia B. Folic acid deficiency C. Hypersplenism D. Acanthocytosis

Hematology and Oncology:Question 6 A 62-year-old man is evaluated because of increasing fatigability. He previously felt

fine. He has recently been found to have osteomyelitis underlying a diabetic foot ulcer and is being treated with ciprofloxacin and amoxicillin/clavulanate. Laboratory studies: Hemoglobin 9.8 g/dL Mean corpuscular volume 79 fL Serum creatinine0.9 mg/dL Serum iron 38 μg/dL Serum total iron-binding capacity 190 μg/dL Serum ferritin 298 ng/mL Which of the following constitutes appropriate additional therapy? A. Cyanocobalamin B. Ferrous sulfate C. Parenteral iron D. Recombinant human erythropoietin and ferrous sulfate Hematology and Oncology:Question 7 A previously healthy, 59-kg (130-Ib), 35-year-old woman delivers her fifth child after an uncomplicated pregnancy and labor. Her blood type is group O, Rh-positive. Soon after the birth, vaginal bleeding is noted that requires emergency transfusion of four units of packed red blood cells. Results of an antibody screen and direct antiglobulin test were negative at 2 months gestation and again when the patient was hospitalized for the delivery. Seven days later, faint scleral icterus is noted. The hemoglobin is 9.5 g/dL (approximately 1 g/dL less than at discharge after delivery). Serum total bilirubin is 2.1 mg/dL, with a direct component of 0.3 mg/dL. Serum lactate dehydrogenase level is 200 U/L, and haptoglobin is 20 mg/dL. Direct antiglobulin (Coombs) testing shows IgG coating the erythrocytes. There is no hemoglobinemia, and the urine is negative for blood. What is the most likely cause of hemolysis in this patient? A. Improper storage of the units of blood prior to transfusion B. Autoimmune hemolytic anemia C. Glucose-6-phosphate dehydrogenase deficiency D. Erroneous transfusion of group A blood E. Hemolytic transfusion reaction Hematology and Oncology:Question 8 A 60-year-old woman is evaluated because of a 1-year history of fatigue. Her menses ended when she was 50 years old, and she has not had any blood loss since that time. She also notes that she is short of breath with exertion. She recalls that a bone marrow biopsy was done some years ago. She is not sure about her family history because her father died when she was young and her mother died of “liver complications at an early age. On physical examination, she has a bronze hue to her skin. The liver is enlarged 3 cm below the right costal margin. She has swelling of the first and second metacarpophalangeal joints of both hands. Laboratory studies: Hemoglobin 13.0 g/dL Hematocrit 40% Serum ferritin 1900 ng/mL Transferrin saturation 70% Fasting plasma glucose 115 mg/dL Serum alanine aminotransferase 80 U/L Serum aspartate aminotransferase 35 U/L

7 °C (101.7 °F).5 g/dL. Febrile nonhemolytic transfusion reaction E. and activated partial thromboplastin time are normal. Follow-up of liver function and iron status D. prothrombin time. Drug-induced bone marrow suppression Hematology and Oncology:Question 10 A 43-year-old man with severe acquired aplastic anemia has not responded to immunosuppressive agents. and blood pressure is 110/70 mmHg. He remains neutropenic and transfusion-dependent for platelets and red cells. hematocrit is 26 %. his temperature is 38. A polymerase chain reaction test of mutations in the HFE gene is performed on the basis of the clinical picture. What is the most likely diagnosis? A. Transfusion-related graft-versus-host disease D. There is dullness at the right lower lung and egophony. Liver biopsy and phlebotomy Hematology and Oncology:Question 9 A 37-year-old man is hospitalized because of fever and right-sided chest pain. At the age of 29 years he was diagnosed with stage III Hodgkin’s disease and treated with multiagent chemotherapy and radiation therapy. Alloimmunization C. He takes thyroid hormone replacement when he remembers and has used fexofenadine as needed for allergic rhinitis for the past 5 years.000/μL. Hypothyroidism D. Relapsed Hodgkin’s disease B. Cytomegalovirus disease B. On physical examination. and the patient is found to be a homozygote for the C282Y mutation. A year ago. and platelet count is 70.A copy of the bone marrow study that she mentioned reveals only trace macrophage iron stores. Which of the following would be prevented by using irradiated cellular blood products for this patient? A. Sepsis C. Liver biopsy B. leukocyte count is 2200/μL. The hemoglobin is 8. Her older brother and father both have a history of thrombosis. They are both cytomegalovirus-seronegative. She had taken oral contraceptives for several years and had one uncomplicated pregnancy but is not taking any medications at present. pulse rate is 112/min. Secondary myelodysplastic syndrome E. Which of the following is the next best step in this patients management? A. he developed mild anemia with no obvious cause. He has an HLA-identical brother who has been cleared as a donor for his planned allogeneic stem cell transplant. Hemolytic transfusion reaction Hematology and Oncology:Question 11 A 40-year-old Chinese woman is evaluated because of pulmonary emboli that developed 1 week after she arrived in the United States after a 16-hour airplane trip. Factor V Leiden and prothrombin G20210A mutation analyses . Results of a complete blood count. She has no other medical problems. He has been having fatigue and recently developed dyspnea on exertion and intermittent chills. Which of the following studies should be done at this time? A. Phlebotomy C.

2 kg (135 Ib).8 g/dL. Group AB whole blood C. The blood bank never received a specimen for type and screen. his hemoglobin was 10. She weighs 61. When he was first evaluated in the emergency department. Acute myeloid leukemia Hematology and Oncology:Question 13 A 45-year-old man with alcoholic cirrhosis is evaluated because of hematemesis from active bleeding due to esophageal varices. What is the most likely cause of this patients pancytopenia? A. and platelet count was 220. He has been noncompliant with his antiretoviral medications because of the side effects. No testing is required Hematology and Oncology:Question 12 A 34-year-old woman is evaluated because of progressive fatigue and recurrent attacks of abdominal pain. Group O whole blood B. Group O fresh frozen plasma D. and pelvis E. Her pulse rate is 110/min and her blood pressure is 110/70 mm Hg. His appetite is poor. Group AB fresh frozen plasma Hematology and Oncology:Question 14 A 24-year-old man who moved to this country from Sweden 15 years ago is evaluated because of severe weakness and shortness of breath. but admits to taking trimethoprim-sulfamethoxazole twice daily for the past week. Two months ago. over the past year.000/μL Reticulocyte count 10% Blood smear Anisocytosis and polychromatophilia Haptoglobin 0 mg/dL Serum lactate dehydrogenase 645 U/L There is high level of hemosiderin in the urine. Laboratory studies: Hemoglobin 6 g/dL Hematocrit 20% Leukocyte count 2500/μL Platelet count 80. predominantly sinusitis. Myelodysplastic syndrome D. What is the most appropriate transfusion product to treat this patients bleeding? A. abdomen. Screening tests for cancer including CT scans of the chest. respectively. . He receives eight units of uncrossmatched group O packed cells over 3 hours. and no pretransfusion blood is available for testing. Testing for a lupus anticoagulant D.000/μL. and her spleen is palpable 4 cm below the costal margin. Autoimmune hemolytic anemia B. He has a 4-year history of HIV infection and has had intermittent infections. leukocyte count was 3900/μL. protein 5. his prothrombin time and partial thromboplastin time were 34 s and 57 s. Protein C. Paroxysmal nocturnal hemoglobinuria E. Aplastic anemia C. She is pale. and antithrombin III measurements C. but his blood pressure remains unstable and his bleeding continues.B.

5 °F). Antithymocyte globulin and cyclosporine E. She does not take any prescription or over-the-counter medications and she has never been ill.7 g/dL. he exhibits extreme pallor. What is the most appropriate choice of blood product to stop this patient’s bleeding? A.8 mgldL Serum vitamin B12 222 pg/mL Serum folate 14 ng/mL What is the most likely diagnosis? A. Drug-induced marrow suppression B. Her pulse rate is 120/min. and the patient receives a transfusion of cytomegalovirusnegative irradiated red blood cells. Long-term transfusion and iron chelation therapy Hematology and Oncology:Question 16 A 63.000/μL Mean corpuscular volume 101 fL Reticulocyte count 0% Peripheral blood smear No abnormalities Serum total bilirubin 3. His conjunctivae are pale. Allogeneic stem cell transplantation from an HLA-identical sister C. Laboratory studies: Hemoglobin 4 g/dL Leukocyte count 2400/μL Platelet count 129.5 °C (99. Oral melphalan and prednisone D. Chest radiography is normal. and a platelet count of 65. His temperature is 37. hematocrit of 35%.000/μL. Platelet concentrates .2 mg/dL Serum indirect bilirubin2. Autoimmune hemolytic anemia Hematology and Oncology:Question 15 A 24-year-old black woman is evaluated at an urgent care clinic because of sore throat.4 °C (103 °F). He has no skin lesions. The remainder of his physical examination is unremarkable. increasing to 33/min when walking.000/μL. 500 mg/kg daily for 4 days B. An emergency complete blood count showed a hemoglobin of 11.5-kg (140-Ib) 29-year-old woman underwent repeat thoracic spinal fusion surgery. temperature of 39. Parvoviral infection C. As they started to close the incision. Bone marrow aspirate and biopsy show scattered plasma cells and almost complete absence of myeloid elements.Cn physical examination. platelet count 17. She lives with her two sisters. broad-spectrum intravenous antibiotic therapy is administered. and severe fatigue of several weeks duration. HIV-induced marrow dysplasia D. On physical examination she has pallor and swollen and red tonsils bilaterally. and reticulocyte count 0%. a leukocyte count of 6300/μL. chills. the surgeons noticed diffuse bleeding from the surgical site that was not easily controlled by cauterization. Her hemoglobin is 6 g/dL. and her blood pressure is 90/50 mm Hg. His respiratory rate is 22/min at rest. Intravenous immunoglobulin. and his blood pressure is 130/89 mm Hg. What is the most appropriate therapy for long-term survival of this patient? A. After throat and blood cultures are obtained. She had extensive bleeding and received a total of 20 units of packed red blood cells. He has generalized shotty lymphadenopathy. leukocyte count 100/μL. The surgery lasted 3 hours and required the use of 7 L of Ringers lactated solution. his pulse rate is 110/min.

Daily administration of granulocyte colony-stimulating factor subcutaneously B. Laboratory findings include a leukocyte count of 3200/μL. Bleeding time B.5 g/dL.000/μL. hemoglobin of 8.000/μL. which has typically been light. Her blood pressure is 120/80 mm Hg. Administer parenteral iron Hematology and Oncology:Question 20 A 67-year-old woman is evaluated because of a 3-month history of dry cough and progressive fevers. platelet count of 142. and serum ferritin level of 375 mg/dL. Administration of androgens E. Administration of vitamin E Hematology and Oncology:Question 18 A 46-year-old woman is referred for preoperative evaluation before undergoing resection of a newly diagnosed glioblastoma. and platelet count is 193. Metastatic breast cancer was diagnosed 6 months ago. Add ascorbic acid D. She was treated 6 months ago with corticosteroids for polymyalgia . including outcomes of previous surgical procedures E. Which of the following is the most appropriate management of this patient? A. she is afebrile. and iron replacement with ferrous sulfate. Cessation of chemotherapy C.5 g/dL. Fresh frozen plasma D. She has total alopecia and a left mastectomy scar. Whole blood C. Her hemoglobin is 12. Platelet aggregation studies D. Change to an iron polysaccharide complex B. Weekly adminstration of erythopoietin subcutaneously D. There has been no change in the patient’s menstrual blood loss.B. She denies having chills but notes intermittent drenching night sweats. Cryoprecipitate Hematology and Oncology:Question 17 A 54-year-old woman is evaluated because of progressively worsening fatigue and inability to sustain her work hours. On rectal examination. The surgeon is particularly interested in an opinion concerning her risk of bleeding. Her serum iron level is 21μg/dL. On physical examination. and she is currently receiving multiagent systemic chemotherapy. On a return visit 3 months later. is started. leukocyte count is 6700/μL. the patient’s hemoglobin level remains unchanged. stool is black but negative for occult blood. with a hemoglobin of 11. Platelet Function Analyzer-i 00 analysis C. Prothrombin time and partial thromboplastin time Hematology and Oncology:Question 19 A 42-year-old woman with rheumatoid arthritis is found be anemic. Which of the following will provide the best estimate of her surgical risk of bleeding? A. Order a serum ferritin test C. The patient claims to be taking ferrous sulfate as directed and reports mild epigastric discomfort and constipation since starting. Medical history. Which of the following is the next appropriate step in this patient’s management? A. hematocrit of 26%. 325 mg three times daily.0 g/dL.

rheumatica. She currently takes prednisone, 5 mg every other day, with reasonable symptom control. She smoked two packs of cigarettes daily for 20 years. Her family history is unremarkable, except that her husband was treated for tuberculosis 20 years ago. On physical examination, her temperature is 38.6 °C (101 .5 °F), pulse rate is 86/min, and blood pressure is 132/80 mm Hg. She is anicteric. Abdominal examination reveals a palpable spleen tip. The remainder of her physical examination is unremarkable. Radiograph of the chest shows a diffuse micronodular pattern confirmed on CT scan of the chest. Abdominal CT scan confirms mild splenomegaly and shows a 1.5-cm periportal lymph node. Blood cultures (including fungal culture), urine cultures, and sputum cultures (including stains for acid-fast bacteria) show no growth. Bronchoscopy with bronchoalveolar lavage is nondiagnostic for infectious or neoplastic disorders. Laboratory studies: Hemoglobin 10.1 g/dL Leukocyte count 12,400/μL Platelet count 625,000/μL Mean corpuscular volume 89 fL Reticulocyte count 1.3% Differential 62% neutrophils, 10% bands, 3% metamyelocytes, 15% lymphocytes, 6% eosinophils, 2% nucleated erythrocytes, 2% monocytes Serum alkaline phosphatase 360 U/L Serum aspartate aminotransferase 112 U/L Serum alanine aminotransferase 178 U/L Serum total bilirubin 1.1 mg/dL Serum indirect bilirubin0.7 mg/dL Serum albumin 3.2 g/dL Skin tests for tuberculosis and Trichophyton are negative; she is anergic. What would be the most appropriate next step in the management of this patient? A. Liver biopsy B. Bone marrow biopsy C. Splenectomy D. Abdominal (periportal) lymph node aspiration Hematology and Oncology:Question 21 A 32-year-old woman with sickle cell disease is evaluated in the emergency department because of diffuse bone and joint pains typical of sickle cell crisis. In the past week, she has noted generalized malaise, a low-grade fever, and mild exertional dyspnea without cough. The patient states that her last painful crisis occurred more than 6 months ago and was treated with rehydration, intravenous narcotic analgesics, and two units of packed red blood cells. On physical examination, the patient appears uncomfortable. Her temperature is 38.4 °C (101.2 °F), pulse rate 108/min, respiratory rate 20/min, blood pressure 126/92 mm Hg. She is mildly icteric. Bibasilar crackles are audible on pulmonary examination. Her abdomen is soft and nontender; there is no palpable organomegaly. Transcutaneous pulse oximetry with the patient breathing room air indicates 95% arterial oxygen saturation. Results of chest radiography are unremarkable. Laboratory studies: Hemoglobin 4.2 g/dL Hematocrit 11 .8% Mean corpuscular volume 88 fL Mean corpuscular hemoglobin 22 pg Mean corpuscular hemoglobin concentration 34 g/dL Leukocyte count 1 2,000/μL Platelet count 200,000/μL

Reticulocyte count 0.2% What is the most likely cause of the patients current status? A. Parvovirus B19 infection B. Alloantibodies against transfused erythrocytes C. Folate deficiency D. Splenic sequestration Hematology and Oncology:Question 22 A 67-year-old man who had peripheral vascular surgery 2 months ago is hospitalized because of substernal chest pain and electrocardiographic changes consistent with myocardial ischemia. His hemoglobin is 12.2 g/dL, leukocyte count is 7300/μL, and platelet count is 235,000/μL. An infusion of unfractionated heparin is initiated, but pain persists intermittently. Two days after admission, his repeat platelet count is 90,000/μL. What is the most appropriate management for this patient? A. Continue the heparin infusion, monitor the platelet count daily B. Continue the heparin infusion and order an ELISA test for heparin-platelet factor IV antibodies. C. Discontinue the heparin infusion D. Add an antiplatelet drug E. Discontinue the heparin infusion and institute thrombin-inhibitor therapy Hematology and Oncology:Question 23 Four days ago, a 30-year-old woman with ulcerative colitis required transfusion with packed red cells for recurrent gastrointestinal bleeding during a flare of her disease. The blood bank distributed group O, Rh-negative units for her because they were the only antigen-negative units available. The antibody screen performed in the emergency department did not show any additional antibodies. This patient has received many red cell transfusions since her diagnosis 10 years ago. Her blood type is group A, and she is Rh-positive. She has a history of positive response to a red cell antibody screen, with anti-Kell, anti-Duffy(a), and anti-E identified. On the day after her present transfusion, the patients hemoglobin level increases by 3 g/dL. The bleeding subsides after high-dose corticosteroid therapy but starts again as the dose is lowered. The patients blood pressure is stable, but her physician requests that additional units be prepared for her in case the bleeding becomes more active. The blood bank refuses the request until a new compatibility sample is sent. The new compatibility sample is needed to detect which of the following? A. New alloantibodies B. A hemolytic transfusion reaction C. Residual group O red cells D. New autoantibodies E. Antibodies missed in the first sample Hematology and Oncology:Question 24 A 55-year-old man who is HIV-positive is evaluated because of weakness, shortness of breath, and a dry, nonproductive cough. He also reports dizziness, cold intolerance, and an inability to continue his regular daily jogging routine. Physical examination reveals pallor, a flow murmur, and bilateral crackles in the lungs. Laboratory studies: Hemoglobin 7 g/dL Mean corpuscular volume 75 fL

Red cell distribution width 17 Reticulocyte count 0.8 % Leukocyte count 8700/μL Serum ferritin 180 ng/mL Serum transferrin 180 mg/dL Serum iron 10 μg/dL A chest radiograph shows an interstitial infiltrate consistent withPneumocystis carinii pneumonia. Which of the following is the most appropriate approach to the anemia? A. Assume the patient has the anemia of chronic disease; direct diagnosis and treatment to the underlying inflammatory process B. Initiate treatment with ferrous sulfate and observe the patients response over the next several weeks C. Treat the patient with erythropoietin D. Perform a bone marrow aspirate and stain with iron stain; if negative, evaluate the gastrointestinal tract for evidence of bleeding Hematology and Oncology:Question 25 In an office visit for an annual checkup, a 46-year-old man reports that he has had malaise and intermittent sweats for the past few months but has been able to continue his job as a high school teacher. Two years ago he was treated for stage III diffuse large-cell non-Hodgkin’s lymphoma with six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) and attained complete remission. He takes simvastatin for hypercholesterolemia and hydrochlorothiazide for hypertension. On physical examination, he has lymphadenopathy: a 3-cm right axillary node and a 2-cm right supraclavicular node. His spleen tip is palpable. Laboratory evaluation shows mild normochromic, normocytic anemia and an elevated serum lactate dehydrogenase level. CT scans of his chest and abdomen reveal additional mediastinal and retroperitoneal lymphadenopathy. What is the best next step in this patient’s management? A. A 2-week course of oral antibiotics B. Referral for salvage chemotherapy and autologous stem cell transplantation C. A repeat physical examination and CT scans in 3 months D. Referral for treatment with investigational agents E. A repeat course of CHOP Hematology and Oncology:Question 26 A 50-year-old woman requests hormone replacement therapy (HRT) for severe hot flashes. Family studies done after her daughter developed deep venous thrombosis in association with use of oral contraceptives showed the patient to be heterozygous for the factor V Leiden mutation. Subsequently, several other members of her large extended family were found to have the factor V Leiden mutation, but none has yet had a thrombotic event, including several older than 65 years. The patient is healthy, and results of a physical examination are within normal limits. Which of the following regimens is most appropriate for management of this patient? A. Initiate HRT along with warfarin, with a target INR of 1.5 to 2 B. Initiate HRT along with enoxaparin and warfarin, with a target INR of 2 to 3 C. Initiate HRT along with warfarin, with a target INR of 2 to 3 D. Recommend against the use of HRT; initiate anticoagulant prophylaxis at times of increased thrombotic risk

Bone marrow aspiration and biopsy B. Her medical history is unremarkable. Function of the allograft has been good with only one episode of mild rejection several weeks after transplant that was successfully managed by changing her immunosuppressive regimen. leukocyte count of 5000/μL. and platelet count of 30. Terminate the pregnancy because of the high risk of thrombosis and obstetric complications E. but no other abnormalities. epistaxis.000/μL. and no abnormalities were identified. or splenomegaly. Prednisone.E. She recently underwent screening for thrombophilia. Measurement of the platelet count of her parents and siblings D. Bone marrow aspiration and biopsy C. Platelet aggregation studies C. Platelet transfusion D. trimethoprimsulfamethoxazole. No intervention. follow-up with monthly platelet counts . Immediately initiate prophylactic doses of low-molecular-weight heparin and continue prophylaxis for 6 weeks after delivery C. Laboratory studies reveal a hemoglobin of 11. Immediately initiate therapeutic doses of low-molecular-weight heparin and continue therapy for 6 weeks after delivery D.2 g/dL and the leukocyte count is 50004iL. Discontinuation of trimethoprim-sulfamethoxazole B. Administration of epsilon-aminocaproic acid E.9 g/dL. The physical examination is within normal limits. she sustained deep venous thrombosis while taking an oral contraceptive preparation. Monitor her pregnancy and initiate low-molecular-weight heparin at prophylactic doses after delivery and continue for 6 weeks B. What is the most appropriate management for this patient? A. tacrolimus. or menorrhagia. Her medical history is otherwise unremarkable and she is not currently taking anticoagulants or other medications. Do not administer anticoagulation before or after delivery unless she develops symptomatic venous thromboembolism Hematology and Oncology:Question 29 A 27-year-old woman is found to have a platelet count of 50. What is the optimal management of this patient? A. There is no family history of venous thrombosis. purpura. and furosemide. She underwent orthotopic cardiac transplantation 4 months ago following the development of postpartum cardiomyopathy. The hemoglobin level is 13. Physical examination shows no petechiae. Discontinuation of immunosuppressive drugs Hematology and Oncology:Question 28 A 25-year-old pregnant woman is evaluated at the end of her first trimester.000/μL. Peripheral blood smear shows reduced platelets but is otherwise normal. A peripheral blood smear shows decreased platelets. Recommend against the use of HRT and prescribe aspirin therapy Hematology and Oncology:Question 27 A 37-year-old woman is referred for evaluation of the new onset of thrombocytopenia. 1 mg/kg per day E. Three years ago. she has not had excessive bruising. Which of the following is the best management recommendation? A. Her current medications include cyclosporine.

Arterial blood gas results are normal. Incidental (gestational) thrombocytopenia D. Hematocrit is 62%.Hematology and Oncology:Question 30 A 61-year-old man is evaluated because of progressive fatigue. On physical examination. Examination of the chest reveals no abnormalities. Cytomegalovirus disease . and platelet count 544. One previous pregnancy resulted in term delivery of a healthy child. What is the best next step in this patient’s management? A. Cytarabine and idarubicin induction chemotherapy D. He takes a cholesterol-lowering agent for hypercholesterolemia. He had successful right hip replacement surgery 3 years ago for severe degenerative arthritis. Phlebotomy C. after which it declined gradually. She has anemia. On physical examination. without much response. Laboratory studies: Hemoglobin 12. The spleen is palpable 3 cm below the costal margin. Preeclampsia E. and has been receiving weekly erythropoietin and intravenous iron for 2 months. leukocyte count 17. There are no family members who are HLA matched as potential donors. Splenectomy B. She is currently in her 31st week of an otherwise uncomplicated pregnancy. Current medications include prenatal vitamins. Idiopathic (immune) thrombocytopenic purpura C. He has no edema. Disseminated intravascular coagulation B. with an oxygen saturation of 97%. but the karyotype does not reveal the presence of the Philadelphia chromosome. Drug-induced thrombocytopenia Hematology and Oncology:Question 32 A 29-year-old woman with systemic lupus erythematosus and end-stage renal disease from glomerulonephritis is dialysis-dependent. his pulse rate is 84/min. He does not smoke but drinks one or two shots of whiskey several days a week.200/μL.000/μL. She is cytomegalovirus-seropositive and has never been pregnant. Which of the following would be prevented by using leukocyte-reduced blood for this patient? A. dyspnea on exertion. Her uterus is appropriately enlarged for gestational age.000 /μL A peripheral blood smear shows only decreased platelets. His bone marrow is hypercellular. Her platelet count was normal until approximately week 26. and headaches over the course of the past 6 months. which are only partially helped by anti-inflammatory therapy. Discontinuation of alcohol intake Hematology and Oncology:Question 31 A 29-year-old woman is evaluated because of thrombocytopenia. She has had no epistaxis or other bleeding complications and no personal or family history of hematologic disease. Her physician wishes to order a transfusion to treat her increasing fatigue. her blood pressure is 120/80mm Hg. He also notices intense itching after he takes a shower. and she is on the waiting list for a cadaveric kidney transplant. She has significant arthritis and polyserositis. His face appears swollen and flushed. She has never received any kind of blood product. Which of the following is the most likely cause of this patients thrombocytopenia? A. and his blood pressure is 145/85 mm Hg.1 g/dL Leukocyte count 8200/μL Platelet count 93.

The leukoerythroblastic blood smear suggests a marrow infiltrative process.400/μL Platelet count 325. 2% monocytes Urine is negative for hemoglobin and positive for hemosiderin. Her parents were both raised in South Africa but have no known history of anemia or other blood dyscrasias. partial thromboplastin time. blood pressure is 155/100 mm Hg. warmth. uncompensated hemolysis. pulse rate is 11 5/min. 3% metamyelocytes. eyes. Weekly dosing of primaquine is preferable to low-dose daily administration.3% Serum total bilirubin 2. The absence of free hemoglobin in the urine excludes recent intravascular hemolysis. HLA alloimmunization C. Two days later. routine serum chemistry studies. D. Which of the following statements about this patients condition is true? A. E. There is no history of recent surgery.6 mg/dL Serum lactate dehydrogenase 667 U/L Differential count 60% neutrophils. she feels short of breath and goes to the emergency department. Rectal examination is unremarkable and a stool specimen is negative for occult blood.3 °C (99.1 °F). Results of a complete blood count. and prostate-specific antigen are normal. His mother had phlebitis in her 80s. Her medical history is otherwise unremarkable. Long-term continuation of primaquine would lead to severe. nose. ears. Hemolytic transfusion reaction Hematology and Oncology:Question 33 A 55-year-old black American woman prepares to tour South Africa and visit relatives. 3% eosinophils. Over the next week she develops weakness and notes dark urine. The patients ethnic background indicates she has a severe enzyme deficiency. No lupus anticoagulant is detected and chest radiograph is within normal limits. Four years ago. The patient is a heavy smoker. and throat examination are unremarkable. Peripheral blood smear shows Heinz bodies and bite cells. she had a cardiac valve replaced for severe aortic stenosis and takes warfarin. The remainder of her physical examination is normal. She has slight icterus. Results of head. travel. compression ultrasound testing reveals deep venous thrombosis extending into the popliteal vein. her temperature is 37.000/μL Mean corpuscular volume 101 fL Reticulocyte count 7. Febrile nonhemolytic reaction E. 15% lymphocytes. Transfusion graft-versus-host disease D. C. Cardiac examination is unremarkable except for mechanical valve sounds. Hematology and Oncology:Question 34 A previously healthy 65-year-old man is evaluated because of a 3-day history of swelling. prothrombin time. B. She begins malaria prophylaxis with primaquine shortly before she is scheduled to leave. or immobilization. Oxygen saturation is 97% with the patient breathing room air.0 mg/dL Serum indirect bilirubin 1.2 g/dL Leukocyte count 1 0. Laboratory studies: Hemoglobin 9. On physical examination. 2% nucleated erythrocytes. . and erythema in the left calf.B. 15% bands.

The peripheral blood smear shows schistocytes and decreased platelets.Which of the following is the next best step in the evaluation of this patient? A. 81 mg/d. Functional assessment of plasma ADAMTS 13 (von Willebrand factor-cleaving protease) activity B. abdomen. Which additional studies are of most use in diagnosing this disorder? A. His only complaint is bloating and fullness after meals. The spleen tip is palpable below the costal margin. Measurement of antibodies against ADAMTS 13 C. pulse rate is 84/min. leukocyte count of 9000/μL. protein C.3 g/dL. previous healthy man presents with a 3-day history of low-grade fever and confusion. CT scan of the chest. Cytogenetic analysis of peripheral blood mononuclear cells C. No further evaluation is required B. his temperature is 36. or carotid or abdominal bruit. but there is no abdominal tenderness. Liver function studies E. Leukocyte alkaline phosphatase score E. CT scans of the chest. There is no occult blood in the stool.3 kg (5 Ib) since last year.000/μL Differential Mostly segmented and band neutrophils and circulating metamyelocytes and myelocytes What is the diagnostic test of choice? A. Blood culture .5 °C (97. Laboratory studies: Hemoglobin 13 g/dL Hematocrit 40% Mean corpuscular volume 90 fL Leukocyte count 37. Measurement of serum lactate dehydrogenase D. abdominal mass. measurements of levels of antithrombin III. He has gained 2. Transesophageal echocardiography D. He has no lymphadenopathy. He takes hydrochlorothiazide for hypertension and aspirin. Physical examination demonstrates pallor and ecchymoses. and protein S) Hematology and Oncology:Question 35 A 50-year-old. Fasting plasma homocysteine level E. On physical examination. upper and lower gastrointestinal endoscopy D. Laboratory studies reveal a hemoglobin of 8. CT scan of the brain Hematology and Oncology:Question 36 A 54-year-old man presents to the office for an annual checkup. and platelet count of 12.000/μL. and his blood pressure is 140/85mm Hg. He feels well and exercises regularly.7 °F). Evaluation for a hereditary thrombotic disorder (tests for factor V Leiden and prothrombin G2021 0A mutations. Peripheral pulses are palpable and normal.000/μL Platelet count 470. CT scan of the abdomen B. and pelvis C.

000/μL. Thrombotic thrombocytopenic purpura Hematology and Oncology:Question 38 An 18-year-old black man is discovered to have a mild microcytic hypochromic anemia during a routine physical examination. Glucose-6-phosphate dehydrogenase deficiency E. prothrombin time and activated partial thromboplastin time are both normal. The patient is unaware of any significant relevant family history. and the patient undergoes an uncomplicated procedure 2 weeks later. Disseminated intravascular coagulation D. Heparin-induced thrombocytopenia E. Sickle cell disease Hematology and Oncology:Question 39 A 78-year-old woman underwent partial colonic resection for repair of a diverticular abscess. Coronary artery bypass grafting is recommended. What is the most likely cause of her thrombocytopenia? A. which shows three-vessel coronary artery disease. Hemoglobin A (α2β2) 95% (normal > 97. Two weeks after discharge she is readmitted to the hospital with a platelet count of . β-Thalassemia trait C. Platelet count at this time is 85. and low-molecular-weight heparin therapy is empirically initiated to treat a presumed pulmonary embolism. the patient’s platelet count is 25.5%) Hemoglobin F (α2γ2 )0% (normal 0%) Hemoglobin S (α21βS2) 0% (normal 0%) Iron studies are pending. Seq uela of bypass surgery C.5%) Hemoglobin A2(α2δ2) 5% (normal <2. During her 2-week hospitalization. The platelet count was 21 5. she received a twice-daily injection of unfractionated heparin.000/μL on admission and remained stable at discharge.4% Mean corpuscular volume 64 fL Mean corpuscular hemoglobin 19 pg Mean corpuscular hemoglobin concentration 30 g/dL Reticulocyte count 4. α-Thalassemia trait D. Iron deficiency B.0% Hemoglobin electrophoresis shows the following results. Four days later she is evaluated in the emergency department because of shortness of breath. She is discharged 3 days after surgery with the platelet count not having been repeated. Idiopathic thrombocytopenic purpura B.000/μL. Physical examination reveals a normal-appearing youth who is anicteric. The following morning.Hematology and Oncology:Question 37 A 67-year-old woman has an uncomplicated myocardial infarction. and radiography of the chest shows no infiltrates. A ventilation-perfusion lung scan is indeterminate.000/μL. Her platelet count immediately before her surgery is 325. She returns to the hospital 2 weeks later for cardiac catheterization.000/μL. What is the most likely cause of this patient’s anemia? A. and the day after is 210. and peripheral blood smear shows only decreased numbers of platelets. the spleen is not palpable. She is afebrile. and the patient has developed acute ischemia of her right foot. Laboratory studies: Hemoglobin 13 g/dL Hematocrit 32.

Continue medications that she takes for her migraine headaches Hematology and Oncology:Question 42 A previously healthy 38-year-old woman is evaluated because of a 2-week history of fatigue and malaise. Which of the following is the best therapy for this patient? A. Her complete blood count and serum chemistry studies are also normal.2 g/dL . Hepatitis C virus C. and carotid ultrasound examination are all within normal limits. Laboratory studies: Hemoglobin 9. Which study is most likely to yield the cause of this patient’s thrombosis? A. which she has had for the past decade. with a target INR of 2 to 3. Results of MRI of the head. Genetic assay for the prothrombin G2021 0A mutation C. She states that she does have occasional bilateral hand stiffness that responds to ibuprofen. Human immunodeficiency virus D. with a target INR of 3 to 4 D. 325 mg/d. 1gM antibody level is normal. There is no lymphadenopathy. and aspirin. Hepatitis B virus Hematology and Oncology:Question 41 A 52-year-old woman is evaluated because of transient numbness on the right side of her face and blurred vision lasting up to 24 hours. Human T-cell lymphotropic virus B.27. Warfarin. Aspirin. Platelet aggregation studies E. but her current symptoms are distinct from those associated with her usual migraines.000/μL and a painful. Warfarin. The cardiolipin IgG is elevated at 50 GPL U/mL (reference range <16 GPL U/mL). and clopidogrel E. She has not had any unexplained weight loss and says she does not have fevers or night sweats. The prothrombin time is within normal limits. 1 4C-serotonin release assay for heparin-platelet factor 4 antibodies D. dusky left lower extremity. transesophageal echocardiography. Aspirin. Results of a lupus anticoagulant panel are positive.1 g/dL and leukocyte count is 6500/μL. Neurologic examination is within normal limits. Review of the peripheral blood smear shows only decreased numbers of platelets and no schistocytes. Hemoglobin is 12. 81 mg/d C. The prothrombin time and partial thromboplastin time are normal. Physical examination shows slight scleral icterus and a mildly enlarged spleen. 325 mg/d B. but the partial thromboplastin time is 55 s (normal limit as high as 34 s). Platelet-associated IgG study Hematology and Oncology:Question 40 What virus is associated with the highest risk of transmission through blood transfusion? A. Physical examination is unremarkable except for livedo reticularis. Arteriogram of the left lower extremity shows a thrombus in the left common femoral artery. Genetic assay for the factor V Leiden mutation B. The patient has a history of migraine headache.

She has not seen a physician for more than 5 years and describes herself as generally healthy. What is the best initial therapy for this patient? A. Eight months ago. She has not had any recent change in weight. Results of iron studies are normal. Erythropoietin Hematology and Oncology:Question 43 A 62-year-old black woman is evaluated after a recent emergency department visit for a transient ischemic attack. her temperature is 36. her pulse rate is 84/min. he had a deep venous thrombosis in his left iliac vein associated with a central venous catheter. He was treated with low-molecular-weight heparin followed by warfarin. She has intermittent dizziness and headaches but not vision changes. and fasting blood glucose 165 mg/dL. Hydroxyurea and low-dose aspirin D. leukocyte count 7200/1iL. His systolic blood pressure is now stable at 100 mm Hg. His prothrombin time is 17 s and partial thromboplastin time is 33 s. He refused colonoscopy at that time. He required a transfusion of four units of blood.8% A peripheral blood smear shows polychromatophilia.5 g/dL. recurrence of gastrointestinal bleeding was detected but has now stopped. What is the most appropriate treatment of this patients disorder? A. and lowmolecular-weight heparin has been administered twice daily since then. Two days ago. Busulfan and aspirin Hematology and Oncology:Question 44 A 55-year-old retired longshoreman is evaluated because he has 3+ edema in both legs. weakness. and no edema.9 g/dL. Stool is negative for occult blood. and surgical resection is planned. Splenectomy E. Intravenous immunoglobulin C.Leukocyte count 8200/μL Platelet count 335. Low-dose aspirin C. Serum albumin level is 1. Liver disease B. Danazol D. he had a recurrent thrombosis in the iliac vein. She has no carotid bruits or cardiac murmur.000/μL Reticulocyte count 9. Two months ago. Prednisone B.000/μL.5 °F). Her bone marrow is hypercellular. and a rare nucleated erythrocyte. What is the most appropriate reason to order transfusion of fresh frozen plasma before this patient undergoes surgery? A. or numbness in her extremities. Colonoscopy reveals an ulcerating polyp. On physical examination.100. Prolonged prothrombin time . Hydroxyurea and warfarin B. Hypoproteinemia D. Hemoglobin is 12. Results of electrocardiogram and chest radiography are normal. fibrosis is not evident and karyotype is normal.4 °C (97. The warfarin was stopped after 3 months because of difficulty regulating his INR and intermittent melena. platelet count 1. Hypovolemia C. no splenomegaly. occasional spherocytes. and her blood pressure is 135/82 mm Hg.

and warfarin. She has had fatigue.000/μL Reticulocyte count 3. 30mg subcutaneously every 12 hours. lethargy. Examination of the peripheral blood smear shows decreased platelets. 1-3 erythrocytes. Laboratory studies: Hemoglobin 7.5% (elevated. Intravenous immunoglobulin C.E. no casts . and dyspnea on exertion for 2 days. Placement of an inferior vena cava filter E. 5 mg/d.000 cells/μL) Serum haptoglobin < 6 mg/dL Serum lactate dehydrogenase 2130 U/L Serum electrolytes Normal Serum creatinine 2. 10 mg for 2 nights. check the patients INR the following day and adjust the dosage if necessary C. Intravenous anti-Rh(D) D. Enoxaparin. adjusted to an INR of 2 to 3. 0 leukocytes/hpf. There is no family history of thromboembolic disease. Which of the following treatments is the most appropriate? A. Observation alone. Low-molecular-weight heparin therapy Hematology and Oncology:Question 45 A 34-year-old woman is evaluated because of easy bruisability. She has previously been in good health with no personal or family history of hematologic disease. absolute count 107. 5 mg/d.000/μL. and blood pressure 110/80 mm Hg. Her temperature is 37.8 °F). leukocyte count is 6200/μL. and platelet count is 18. Ibuprofen. Hematology and Oncology:Question 47 A 43-year-old previously healthy woman is evaluated because she develops a severe headache and becomes confused. On physical examination. What is the most appropriate management for this patient? A. Her hemoglobin is 12. Her conjunctivae and mucous membranes are pale. with monthly platelet counts B. Enoxaparin. pulse rate 93/min.3 g/dL Hematocrit 23% Leukocyte count 6500/μL Platelet count 37. He has no other medical problems and is a nonsmoker. for which he takes an angiotensin-converting enzyme inhibitor. She has no hepatosplenomegaly. Prednisone Hematology and Oncology:Question 46 A 45-year-old man develops symptomatic deep venous thrombosis of the left leg 1 week after arthroscopic surgery.8 g/dL. and warfarin. occasional petechiae are evident over the distal lower extremities. Prednisone followed by immediate splenectomy E. with some platelets slightly increased in size. Doppler ultrasound examination shows a thrombus in the left posterior tibial vein extending to within 2 mm of the popliteal vein.3 mg/dL Blood urea nitrogen 43 mg/dL Urinalysis 3+ blood. Neurologic examination reveals a confused and disoriented patient without focal findings. and obtain a repeat ultrasound in 1 week B. 1 mg/kg body weight subcutaneously every 12 hours. adjusted to an lNRof 2 to 3 D.7 °C (99. 600 mg every 8 h. Warfarin. The patient has mild hypertension.

7 g/dL and his hematocrit is 32%. Begin therapeutic doses of low-molecular-weight heparin. Hospitalize the patient for intravenous administration of heparin when his INR is less than 2. Broad-spectrum antibiotics are administered intravenously. stop the heparin before surgery and administer anticoagulant prophylaxis for 1 week after surgery D. and a diagnosis of acute myocardial infarction is considered. His hemoglobin is 9. His left ventricular ejection fraction is 35% by echocardiogram. nonbloody diarrhea for 3 . but his respiratory status worsens and he requires intubation and ventilatory support. and his systolic blood pressure has ranged from 70 to 110 mm Hg.35. Hypovolemia Hematology and Oncology:Question 50 A 44-year-old man is evaluated because he has had foamy. Plasmapheresis with fresh frozen plasma as replacement fluid C. He also has fever and dyspnea. Ventilatory support C. Platelet transfusion D. resume therapeutic doses of low-molecular-weight heparin 12 hours after surgery E.2 mg/dL on admission. Plasmapheresis with saline and albumin as replacement fluid B. Oxygen saturation is 94% while breathing 50% inspired oxygen of 50%.Results of a direct antiglobulin test are negative. and chest radiograph shows lung infiltrates consistent with aspiration pneumonia. The laboratory identifies polychromasia. Administer antithrombotic prophylaxis for 1 week after surgery B. Arterial blood gas measurement shows a Po2 of 75 mm Hg. His serum albumin is 2. and nucleated erythrocytes on a peripheral blood smear. He takes no medications except warfarin. What is the best initial therapy for this patient? A. 325 mg/d postoperatively for 10 days Hematology and Oncology:Question 49 A 69-year-old homeless man is hospitalized because of chest pain and electrocardiographic changes. Hem atocrit below 36% B. numerous schistocytes (fragmented erythrocytes). High-dose corticosteroids Hematology and Oncology:Question 48 A 57-year-old man is referred by his orthopedist for recommendations regarding anticoagulation therapy before knee replacement surgery next month. Use pneumatic compression boots during and after surgery and start aspirin therapy. In addition to stopping the warfarin. Hypotension E. and give a prophylactic dose the morning of surgery. Provide adequate antithrombotic prophylaxis after surgery and continue anticoagulation for a total of 6 weeks C. Prothrombin time and partial thromboplastin time are normal. which of the following anticoagulation regimens is most appropriate for this patient? A.0. His pulmonary capillary wedge pressure is below normal. What is the most appropriate reason to order packed red cell transfusion for this patient? A. He had an unprovoked deep venous thrombosis 6 months ago. Hypoxia D. and pH of 7. Pco2 of 35 mm Hg.

Stool culture shows no growth of enteric pathogens. There is no abdominal tenderness. He has a history of chronic myeloid leukemia. On physical examination. she required a caesarean section.1 mg/dL. chest. for the past year and aspirin. leukocyte count of 45004iL. Chronic graft-versus-host disease D. Discontinue oral anticoagulation and counsel her about prophylactic anticoagulation in high-risk situations D.8% Platelet count 87. She is afebrile. She was hospitalized for 2 weeks before the delivery because of preeclampsia. Neutrophils have reduced segmentation and granulation.weeks and has lost 4. and back. Shigella enteritis Hematology and Oncology:Question 51 A 40-year-old woman developed deep venous thrombosis and pulmonary embolism 1 month post partum. Pseudomembranous colitis B. and there is pseudo-Pelger-Huet hypolobulation. loperamide. for more than 10 years. She has been taking ramipril.5 g/dL Leukocyte count 2200/μL Mean corpuscular volume 105 fL Reticulocyte count 0. Resume oral anticoagulation therapy for an indefinite period at a target INR of 2 to 3 B. she too is found to have protein S deficiency. including at night.5 kg (10 Ib). Laboratory results show a hematocrit of 33%. 5 mg/d. After 6 months of anticoagulation. He is taking prednisone. two to three times daily. and generally does not feel well. 10 mg/d. Two weeks later.000/μL Peripheral blood smear shows ovalomacrocytes and a few nucleated erythrocytes with nuclear budding. . Administer low-molecular-weight heparin and resume anticoagulation with warfarin at a fixed dose of 1 mg/d for 2 years C. splenomegaly. and trimethoprim-sulfamethoxazole twice a week. He reports five to six bowel movements a day. and there are lichenoid changes over the skin of his arms. and total serum bilirubin of 1. She has had progressive fatigue and poor appetite. or lymphadenopathy. for which he underwent allogeneic stem cell transplantation 9 months ago. Which of the following is appropriate therapy for this patient? A. Laxative abuse C. His diarrhea is associated with crampy abdominal pain. Her brother had an episode of venous thrombosis at the age of 33 years and was found to have protein S deficiency. Laboratory studies: Hemoglobin 7. her warfarin therapy is discontinued and coagulation studies are done. and her pulse rate is 100/min. Initiate treatment with aspirin and clopidogrel Hematology and Oncology:Question 52 A 74-year-old woman with a history of diverticulosis and hypertension presents for an urgent office visit. On physical examination. 81 mg/d. Warfarin therapy is begun after delivery. she is pale. platelet count of 110. What is the most likely cause of his diarrhea? A. Her father was also found to have a protein S deficiency but is in his 60s and has not had venous thromboembolism. He has generalized muscle atrophy. he has generalized hyperpigmentation.000/μL. Recurrence of chronic myeloid leukemia E.

9 °C (102 °F). She receives daily injections of unfractionated heparin for prophylaxis of venous thrombosis. including a platelet count of 1 5. and the blood bank releases group AB platelets for his transfusion. Drug-induced aplastic anemia C.000/μL. Initiate warfarin immediately after discontinuing argatroban B. On admission her hemoglobin is 11. which is titrated to a final dose of 3 μg/kg per minute to maintain a stable partial thromboplastin time of 2 X control. Myelodysplastic syndrome D. and discontinue argatroban when the INR is greater than 4. A hematology consultant recommends initiating systemic anticoagulation therapy with argatroban after the result of a heparinplatelet factor 4 enzyme-linked immunosorbent assay is positive. Iron deficiency anemia B.000/μL and a proximal right lower extremity deep venous thrombosis is documented by venous ultrasound. ABC incompatibility D. Initiate warfarin at a loading dose of 10 mg/d.9 g/dL. the platelet count has fallen to 105. Initiate warfarin 24 h after discontinuing argatroban C. and discontinue argatroban when the INR is greater than 2. the patient develops severe hypotension. What is the most likely cause of this adverse transfusion reaction? A. his temperature is 38. leukocyte count is 6500/μL. A pulse oximeter evaluation shows 93% saturation.0 D. What would be the best schedule for discontinuing this patient’s agatroban therapy and initiating warfarin therapy? A. Vascular collapse and acute renal failure follow within a few hours. and no wheezing or lymphedema is noted. and platelet count is 286.0 E. and he has rigors and slight dyspnea. On day 6 of hospitalization. Compression ultrasound shows .0004/μL and an absolute neutrophil count of 600/μL (normal is greater than 2500/μL). The patient’s blood type is known to be group A. Anaphylaxis C.What is the most likely diagnosis? A. despite intravenous administration of fluids and the use of pressors. Donor leukoagglutinins Hematology and Oncology:Question 54 A 68-year-old woman is hospitalized for right total hip replacement. Bacterial contamination B. Argatroban therapy is initiated at a dose of 2 μg/kg per minute. Vitamin Bl2deficiency E. Initiate warfarin at 5 mg/d. A half-hour after the transfusion. He has myelodysplastic syndrome and low blood counts. Myelofibrosis Hematology and Oncology:Question 53 A 65-year-old man is hospitalized because of an episode of epistaxis that lasted longer than 1 hour and some rectal bleeding. Initiate warfarin at 5 mg/d. and discontinue argatroban when the partial thromboplastin time is greater than 4 X control Hematology and Oncology:Question 55 A 60-year-old woman is evaluated because her left leg has become warm and swollen 3 weeks after total right hip replacement. Patient leukoagglutinins E. The patient receives a transfusion of a pool of five platelet concentrates over 30 minutes through a leukocyte-reduction filter.

Desferrioxamine therapy. HELLP syndrome E.5% Serum ferritin 1800 ng/mL Transferrin saturation 40% Serum alanine aminotransferase 60 U/L Serum aspartate aminotransferase 40 U/L Serum bilirubin (total) 2. On physical examination his liver is palpable 3 cm below the right costal margin. Diagnostic liver biopsy to include staining for iron and determination of quantitative iron concentration C. a 37-year-old woman has severe vaginal bleeding from uterine atony. along with 4 L of normal saline. He has had anemia since childhood but has never received a blood transfusion. and uterine curettage. What is the most likely cause of the 5000/μL platelet-count increment? A. and she receives a five-pack of pooled platelet concentrates. Platelet count 10 min after transfusion is 60. He drinks wine regularly in the evening with his meal but denies excessive use of alcohol. The bleeding is unresponsive to uterine massage. Gestational thrombocytopenia C.000/μL. Laboratory studies: Hemoglobin 10. 50 mg/kg body weight daily by subcutaneous infusion over 10 hours . and the plasma fibrinogen level is 330 mg/dL. Bone marrow study to include staining the aspirate with Perls’ reagent D.000/μL. What would be the most appropriate next step in managing this patients presumptive iron overload? A. 6 Weeks B.000/μL before delivery and is now 55. Her prothrombin time and partial thromboplastin time are normal. Active bleeding B. Her platelet count was 160. 3 Months C. 1 Year D. She was treated postoperatively with prophylactic doses of low-molecular-weight heparin for 1 week. Phlebotomy to remove one unit of blood weekly until the serum ferritin level is less than 20 ng/mL B. An emergency hysterectomy is planned. adminstration of prostaglandins.proximal deep venous thrombosis of the symptomatic leg. Disseminated intravascular coagulopathy D. For the rest of her life Hematology and Oncology:Question 56 After delivery of her sixth child.1 mg/dL with an elevated indirect fraction Serum haptoglobin Decreased Serologic studies for hepatitis B and C Negative Results of a test for the C282Y mutation in the HFE gene are negative. How long should the therapy be continued? A. She weighs 70 kg (154 Ib).2 g/dL Mean corpuscular volume 70 fL Reticulocyte count 1. Alloimmunization Hematology and Oncology:Question 57 A 55-year-old man of Greek ancestry is evaluated because of fatigue. She receives 12 units of packed red cells and four units of fresh frozen plasma over 3 hours.

furosemide. atrial fibrillation. She takes ramipril. and a nonsteroidal anti-inflammatory drug. her pulse rate is 72/min.000/μL Serum aspartate aminotransferase 120 U/L Serum total bilirubin 2. Administration of intravenous immunoglobulin B. aspirin. diet-controlled diabetes mellitus. Administration of antiplatelet agents Hematology and Oncology:Question 60 A 74-year-old woman presents for an urgent office visit. Refer her to a bone marrow transplant center E. Plasma exchange D.7 °F).4 kg (12 Ib). her blood pressure is 110/70 mm Hg. Hospitalize her to start induction chemotherapy B.000/μL Differential Mostly segmented and band neutrophils and circulating metamyelocytes Bone marrow aspirate smear shows a hypercellular specimen with a very high myeloid:erythroid ratio. What is the best next step in the treatment of this patient? A. Administer interferon-alfa therapy D. Karyotypic analysis shows a translocation between chromosomes 9 and 22 in all 20 metaphases. and 2% blasts. She has a history of congestive heart failure. a significant left shift. and her blood pressure is 105/70mm Hg. She reports dyspnea on exertion and bilateral knee pain. aspirin (81 mg/d). There is no occult blood in her stool.5 °C (97. and degenerative arthritis. She has lost 5. She has lost 2. and simvastatin. On physical examination. thyroid hormone replacement.Hematology and Oncology:Question 58 A 74-year-old woman has an annual checkup. She has decreased bowel sounds and mild rebound tenderness in the right upper quadrant. The spleen tip is palpable below the costal margin. She has been taking ramipril. Initiate therapy with imatinib mesylate (Gleevec®) Hematology and Oncology:Question 59 A 35-year-old woman is evaluated in her 36th week of pregnancy because of the gradual onset of severe right upper quadrant abdominal pain. She also reports dyspnea on exertion. On physical examination. Discomfort is worse during meals and prevents her from eating full portions. hypothyroidism.3 kg (5 Ib) since last year. digoxin. Aggressive diuresis E.6 g/dL Leukocyte count 4600/μL with 80% neutrophils Platelet count 35. Urgent delivery of the fetus C. .1 g/dL Urinalysis No protein The peripheral blood smear shows abundant schistocytes and decreased platelets. She has been experiencing epigastric discomfort over the last 3 months.000/μL Platelet count 370. her temperature is 36. Laboratory studies: Hemoglobin 11 . What is the most appropriate therapy for this patient? A. Refer her for hospice care C. Laboratory studies: Hemoglobin 9.5 g/dL Hematocrit 36% Leukocyte count 55.

Testing shows that she is heterozygous for the prothrombin G2021 0A gene mutation. The presence of the prothrombin gene mutation clearly identifies her as being at higher risk for recurrent deep venous thrombosis than patients without a prothrombotic defect . Bone marrow cannot be aspirated. and teardrop cells. Use of an angiotensin-converting enzyme inhibitor Hematology and Oncology:Question 62 A 40-year-old woman has an unprovoked first episode of deep venous thrombosis. She has no family history of thrombosis. and her first-degree relatives should be tested for the mutation C. She is group A. Her only medication is ramipril for hypertension. Disseminated tuberculosis E. Rh-positive.000/μL.5 g/dL. Her risk of recurrence is less than 5% per year B. she develops an erythematous. mean corpuscular volume 89 fL.200/μL. Myelodysplastic syndrome D. and face with generalized urticaria and some lymphedema of the oral mucosa. Her hemoglobin is 7. Analysis of blood is negative for t(9. and she complains of crampy abdominal pain and nausea. Thirty minutes after starting infusion of the first unit. She is afebrile and has no back pain. Transfusion-related acute lung injury E. Which of the following is the most important consideration to relay to this patient? A. What is the most likely cause of this transfusion reaction? A. her pulse rate is 88/min. but she has no other biologic defects predisposing her to thrombosis. Bacterial contamination B. The patient has a tooth abscess and requires oral surgery. and her blood pressure is 155/85 mm Hg. There is mild epigastric tenderness with palpation. What is the most likely diagnosis? A. Her hematologist orders four units of fresh frozen plasma to be given just before surgery. ABC incompatibility D. The edge of her liver is also palpable 6 cm below the costal margin. Her spleen is massively enlarged and palpable at the umbilicus. The prothrombin gene mutation is a risk factor for an initial episode of deep venous thrombosis. Results of urinalysis are normal. Hairy cell leukemia C. Wheezing and stridor develop. Her systolic blood pressure is 90 mm Hg. myeloid precursors. The units are group AB. and platelet count 114. trunk. Anaphylaxis C.22) by fluorescent in situ hybridization (FISH). Myelofibrosis Hematology and Oncology:Question 61 A 50-year-old woman has factor XI deficiency (2% activity) and a history of menometrorrhagia and of excessive bleeding during previous foot surgery. Chronic myeloid leukemia B. pruritic rash over her arms.On physical examination. Rh-positive. Peripheral blood smear shows numerous erythroblasts. leukocyte count 11. and biopsy specimen shows marked fibrosis.

It is related to the tamoxifen given to control the breast cancer D. and 5 years of tamoxifen therapy. She underwent splenectomy 18 months ago. The mass is excised and found to be a fibrosarcoma. and her platelet count initially improved. Her hemoglobin is 11.000/μL. Danazol Hematology and Oncology:Question 64 A 68-year-old woman is evaluated because of a lump on her chest wall. The lump she now has is separate from the breast. What is the best next step in this patient’s management? . Cyclophosphamide C. and leukocyte count is 8200/μL. Intravenous contrast perfusion of the two nodules during the CT scan is not suspicious for hemangioma. Bone scan and CT scan of the head are normal. There are no significant findings on physical examination. Pulmonary function tests show mild obstructive disease. Positron emission tomography scan shows distinct uptake in the left perihilar mass but only faint focal activity in the right lobe of the liver. Intravenous immunoglobulin B. but CT scans of the chest and abdomen show the mass and two 1-cm nodules in the right lobe of the liver. and all blood studies are normal. her platelet count began to fall and currently is 12. had been reinitiated without significant improvement in her platelet count. however.8 mg/dL. fixed to the underlying 4th rib. Intravenous anti-Rh(D) E. What is the relationship of this fibroscarcoma to her original cancer and its treatment? A. Prednisone.D. Seventeen years ago she developed stage I ductal adenocarcinoma (estrogen receptor-positive) of the left breast. A trial of which of the following therapies would NOT be appropriate for this patient? A. and bronchoscopic biopsy confirms poorly differentiated adenocarcinoma. It is not related to the original cancer or its treatment B. Her blood type is group B and she is Rh-positive. subcutaneous in location.000/μL. She should take warfarin at a target INR of 2 to 3 for the next 2 years Hematology and Oncology:Question 63 A 44-year-old woman with idiopathic thrombocytopenic purpura is evaluated because of worsening thrombocytopenia. 30 mg/d. Chest radiograph shows a 3-cm mass in the left perihilar region. breast radiation therapy. It is related to the primary estrogen receptor-positive breast cancer Hematology and Oncology:Question 65 A 70-year-old man with an 80-pack-year smoking history is evaluated because of a chronic cough of 6 months duration. She responded for several months to oral corticosteroid therapy but ultimately required more than 60 mg/d of prednisone to maintain her platelet count above 10.000/μL. She presented 3 years ago with menorrhagia and a platelet count of 10. Approximately 6 months ago. and nontender. It is related to the radiation therapy given to control the breast cancer C. Rituximab D. her disease was managed with lumpectomy.

6-kg (1 0-Ib) weight loss over the past several months. His clinical stage is Tic (that is. clinically organ-confined and detected by screening serum prostate-specific antigen [PSA] blood test only). CT scan of the abdomen shows dilation of the common bile and pancreatic ducts. Avoidance of alcohol abuse B. CT-directed liver biopsy D. A fine-needle aspirate of the mass reveals atypical cells that are suspicious for malignancy. He recently noticed that the color of his urine was darker than usual. Her mother died of lung cancer at the age of 60 years. but quit smoking 4 years ago. and her father has had a head and neck cancer. In addition to avoidance of tobacco. Serum carcinoembryonic antigen test E. Urinary incontinence B. loss of appetite. Thrombosis D.2 g/dL Serum aspartate aminotransferase 105 U/L Serum alanine aminotransferase 95 U/L Urinalysis shows elevated bilirubin. and biliary bypass B. Which of the following side effects is he most likely to have with radiation therapy? A. which of the following approaches has been demonstrated to decrease risk for one or both of these cancers? A. as much as possible. Refer the patient for exploratory laparotomy. His PSA is 7 ng/mL and his Gleason score is 6. Impotence C.A.3 g/dL Serum albumin 3. and a 4. Avoidance of exposure to benzene D. He has hypertension that is controlled by medical management. biopsy. Laboratory studies: Hemoglobin 11. He has decided to undergo local therapy for his prostate cancer.2-cm mass in the head of the pancreas. Request a CT-guided biopsy of the mass in the head of the pancreas to establish a diagnosis .5 g/dL Serum total bilirubin 3. Chemotherapy C. a 5. and compression of the superior mesenteric vein. Difficulties with bowel function Hematology and Oncology:Question 68 A 69-year old black man is evaluated because of a history of steadily increasing upper abdominal pain. Radiation therapy B. She is very worried about getting cancer and wants to know what she can do to reduce her risk. What is the most appropriate course of action? A. He has met with a radiation oncologist and a urologist and has decided to undergo external-beam radiation therapy. Daily intake of antioxidant vitamins C. Surgery Hematology and OnCology:Question 66 A 25-year-old woman has her first routine check-up. Daily intake of β-carotene Hematology and Oncology:Question 67 A 66-year-old man has newly diagnosed adenocarcinoma of the prostate gland. of getting either of these cancers. The patient had a 40-pack-year history of cigarette smoking.

but no discrete mass. The patient had been previously healthy and has never smoked. The lymph node is completely resected. Hematology and Oncology:Question 71 A 42-year-old woman is evaluated because she has noticed a thickening in her left breast over the past few weeks. D. Human papillomavirus DNA test for cervical cancer every year C. C. B. She is premenopausal. Chest radiography every year in cigarette smokers for lung cancer Hematology and OnCology:Question 70 A 45-year-old woman is evaluated because of a palpable 2-cm right axillary lymph node. Bilateral mammography is negative. Radiation therapy to the right axilla is required with fields encompassing the right breast. and histologic study shows adenocarcinoma. there appears to be some asymmetry in breast tissue density in the upper outer quadrant of the left breast compared with the right one. CT scans of the chest and abdomen are negative for tumor. When she was 35 years.C. Lung cancer is the most likely diagnosis. MRI of the breast D. but because it seems to come and go. There are no palpable lymph nodes. consultation with a surgeon experienced in breast diagnosis B. hormone receptors are negative. Refer the patient to a tertiary-care center with surgical expertise in the management of patients with pancreatic and hepatobiliary disease Hematology and OnCology:Question 69 Which of the following cancer screening tests has been shown in randomized trials to decrease the risk of death from the target cancer? A. Obtain a consultation for endoscopic retrograde cholangiopancreatography and possible biopsy and biliary stent placement D. Results of bone scan and MRI of the head are also unremarkable. She noticed it a few months ago. What would be the most appropriate next step in this patients management? A. She has no known radiation exposure and no family history of breast cancer. On physical examination. She had menarche at age 13 years. including CA15-3 and carcinoembryonic antigen . and the patient requires careful monitoring for possible future recurrence. Results of a mammogram are negative. Which of the following statements about this patients condition is correct? A. Pap smear of the cervix every year in sexually active women for uterine cancer D. Breast cancer is the most likely diagnosis. Fecal occult blood testing every 2 years for colorectal cancer B. and no further lymphadenopathy is detected. as is MRI of the right breast. and optimal therapy for lung cancer paradigm should be initiated. Breast ultrasound. Reassurance that because her family history is negative and the mammogram is negative. All known disease has been resected. she put off seeking medical attention. Measurement of circulating tumor markers. and optimal therapy for breast cancer paradigm should be initiated. no additional studies or treatment is required C. she had one child (whom she breast-fed) after a normal first full-term pregnancy. She took oral contraceptives for 10 years before her pregnancy.

Radiation therapy with strontium-89 . He was treated 15 years ago for stage IIB massive mediastinal Hodgkin’s disease. a urine culture is also ordered. Pulmonary fibrosis D. the patient noted fatigue and rib pain. Biopsy specimen showed adenocarcinoma of the prostate gland. his clinical condition is otherwise stable. Chest radiograph shows no abnormalities. His Gleason score was 7. Add granulocyte colony-stimulating factor E. Blood samples are obtained from a peripheral vein and through the port and sent for culture. Treatment included doxorubicin. Androgen ablation (medical or surgical) D. vinbiastine. Remove the central venous port Hematology and Oncology:Question 73 A 43-year-old man is evaluated because of crushing substernal chest pain that developed during a pickup basketball game with colleagues at work. and dacarbazine (ABVD) followed by mantle-field radiation therapy to a total dose of 4400 cGy. Results of blood and urine cultures are negative. but there are no localizing symptoms to suggest a source of infection. He has an indwelling central venous port. One month ago. His temperature is 38. the absolute neutrophil count is 165/μL and the platelet count is 56.8 °C (100 °F) and absolute neutrophil count is 4504/μL. A repeat chest radiograph is normal. Switch to oral ciprofloxacin and amoxicillin-clavulanic acid D.Hematology and Oncology:Question 72 A 26-year-old man with testicular cancer who is receiving chemotherapy is evaluated in the emergency department. bleomycin. He has had no recurrence of his Hodgkin’s disease. Myocardial infarction C. and he complains of feeling flushed and tired. He was treated with external-beam radiation therapy. Results of other laboratory studies and urinalysis are within normal limits. Continue the current antibiotic regimen B. What is the most likely diagnosis? A. The patient is hospitalized and intravenous ceftazidime is initiated. the patient’s temperature is 37. Anemia associated with secondary myelodysplasia evolving to acute leukemia E. He takes thyroid medication because he developed hypothyroidism 2 years after completing his therapy for Hodgkins disease.9 gIdL. Constrictive pericarditis Hematology and Oncology:Question 74 Four years ago. Three days later. The patients hemoglobin is 8. Chemotherapy with docetaxel B. and blood and urine specimens are again sent for culture. and bone scan revealed diffuse metastatic disease. Chemotherapy with mitoxantrone and prednisone C.000/μL. Recurrent Hodgkins disease B. a 67-year-old man had a serum prostate-specific antigen (PSA) level of 16 ng/mL. What is the most appropriate treatment strategy for this patient? A. Add vancomycin C. What is the best treatment for this patient? A.9 °C (102 °F). His PSA was found to be 87 ng/mL.

and restaging scans afterward showed marked shrinkage of the right perihilar mass. Neurologic examination is unremarkable. and his only other symptom is mild fatigue. Flexible sigmoidoscopy shows a mass 11 cm from the anus. He has been . Her calculated risk of breast cancer is 10. and distention that have worsened over the past week. but is nonetheless concerned about heart disease. Her mother died of breast cancer. What is the most appropriate next step in this patient’s management? A. Three years ago. It is relieved with ibuprofen. Gallium scan B. annual flexible sigmoidoscopy B. The patient completed combined chemotherapy and radiation therapy 6 months ago. No lymph nodes are involved. Plain radiograph of the thoracic and lumbar spine shows no abnormalities other than signs of mild osteoarthritis. The primary tumor. Which of the following options is reasonable to consider for this patient? A. repeated every 3 to 5 years Hematology and Oncology:Question 76 A 58-year-old postmenopausal woman has been taking hormone replacement therapy with combined estrogen and progestin for the past 4 years because she was told it would decrease her risk for heart disease. Chemotherapy with 5-fluorouracil and leucovorin followed. Postoperative adjuvant chemotherapy and pelvic radiation therapy C. Continue hormone replacement therapy and add tamoxifen C. he was diagnosed with stage III rectal cancer (primary tumor and renal involvement) and underwent low anterior resection. No chemotherapy or radiation therapy. and biopsy reveals adenocarcinoma. and a low anterior resection is performed.Hematology and Oncology:Question 75 A 68-year-old woman is evaluated because of rectal bleeding that began recently and a sense of fullness in the rectum. The patient is considering chemoprevention for breast cancer. Re-evaluation if the symptoms get worse C. Intravenous dexamethasone and MRI of the spine Hematology and Oncology:Question 78 A 72-year-old man is evaluated because of constipation. Recently. Discontinue hormone replacement therapy and start tamoxifen D.4% over the next 5 years. and she is concerned that she is also at risk. The patient is referred to a colorectal surgeon. What is the most appropriate next step in the management of this patient? A. abdominal pain. She has not had a hysterectomy. and pelvic radiation was given with concurrent infusion of 5-fluorouracil. her sister has been diagnosed with breast cancer. CT scan of the chest and abdomen with bone windows of the spine D. He has no muscle weakness. MRI of the spine E. the patient had a breast biopsy that showed atypical hyperplasia. Her father died at age 65 years of heart disease. Continue hormone replacement therapy alone for primary prevention of heart disease B. Two years ago. Change her hormone replacement therapy to estrogen alone and add tamoxifen Hematology and Oncology:Question 77 A 68-year-old man with locally advanced non-small-cell lung cancer is evaluated because of the new onset of low back pain over the past 2 weeks. a 4-cm moderately differentiated adenocarcinoma. Postoperative pelvic radiation therapy D. penetrates the bowel wall. Complete colonoscopy within the first year.

On physical examination. Plain radiograph of the abdomen shows distended loops of small bowel. Chest radiograph shows several lung nodules and infiltrates. No further management C. She has not had a hysterectomy. Squamous cell carcinoma E. Bronchoalveolar cell carcinoma B. his pulse rate is 100/min. Which of the following surveillance strategies for the detection of endometrial cancer is most important to incorporate into this patient’s care? A. a slightly distended abdomen with hyperactive bowel sounds. Annual endometrial aspiration sampling D. what is the most appropriate management of this patient? A. Positron emission tomography now and annually B. and she remains amenorrheic. and some guarding to deep palpation in the left lower quadrant. and she is taking tamoxifen. She received chemotherapy for 6 months. The patient reports no weight loss. Positron emission tomography scan of the abdomen D. Complete colonoscopy B. carcinoembryonic antigen) . He has orthostatic hypotension. and results of his most recent colonoscopy (1 year ago) were unremarkable. What is the next step in this patient’s management? A. Annual transabdominal pelvic ultrasound C. Upper gastrointestinal series with small-bowel follow-through C. Her hair is growing back. her energy is returning. Annual transvaginal ultrasound B. In addition to routine follow-up. Carcinoid tumor Hematology and OnCology:Question 81 A 45-year-old woman has recently undergone lumpectomy and radiation therapy for a stage II breast cancer. The patient most likely has which of the following histologic types of lung cancer? A. Biennial dilatation and curettage E. with no stool in the distal colon or rectum. The patient is hospitalized for bowel rest and intravenous hydration. Blood studies show no abnormalities. Small-cell lung cancer C. Her periods ceased while she was receiving chemotherapy. CT scans confirm pulmonary involvement only. but she is worried about recurrence. There are no significant findings on physical examination. Her tumor was rich in estrogen receptor.having regular bowel movements. Annual routine bimanual pelvic examination Hematology and Oncology:Question 80 An 82-year-old woman who has never smoked is evaluated because of a persistent cough. CT scan of the abdomen with oral and intravenous contrast Hematology and Oncology:Question 79 A 60-year-old postmenopausal woman at elevated risk for breast cancer is taking tamoxifen to reduce her risk. and results of bone scan and CT scan of the head are normal. Large-cell carcinoma D. and she has no specific complaints. Measurement of serum carcinoembryonic antigen E. Routine tumor marker evaluation every 3 to 4 months (serum CAl 5-3.

vincristine. He now has fatigue and early satiety. One month ago. She has fatigue. Total gastrectomy followed by radiation therapy Hematology and Oncology:Question 84 A 65-year-old woman has a modified radical mastectomy for a 1. The patient’s laboratory studies show relatively normal organ function. and has noticed a 2.3-kg (5-Ib) weight loss over the past 3 months. Combination chemotherapy with 5-fluorouracil. Eradication of Helicobacter pylon E.0-cm. She has stopped participating in weekend bike trips with a cycling club. and pelvis E. The tumor is positive for estrogen and progesterone receptors and negative for HER2. Second-line chemotherapy regimen with leucovorin and oxaliplatin D. and mitomycin C (FAM) B. What is the best treatment for this patient at this time? A. doxorubicin.D. and leucovorin. Chest wall radiation therapy and anastrozole for 5 years . Estrogen replacement therapy Hematology and Oncology:Question 82 A 66-year-old black woman diagnosed with stage III colon cancer underwent definitive resection of the primary tumor 3 years ago. She is otherwise healthy. disease progression was documented on restaging CT scans. Bone scan and annual CT of the chest. Metastatectomy B. she received adjuvant chemotherapy with 5-fluorouracil and leucovorin. and prednisone (CHOP) C. Four cycles of CHOP followed by involved-field radiation therapy D. Abundant Helicobacter pylon organisms are noted on biopsy. and histologic evaluation of the mass lesion shows it to be a gastric lymphoma of mucosa-associated lymphoid tissue (MALT) type. Hepatic arterial infusion C. doxorubicin. but remains involved in church and family activities and states that she wants to be as aggressive as possible in fighting the cancer. Chest wall radiation therapy and tamoxifen for 5 years B. What is the most reasonable recommendation for this patient? A. and had a partial response to therapy. High-dose chemotherapy with autologous peripheral stem cell transplantation Hematology and Oncology:Question 83 A 68-year-old man is evaluated because of worsening chronic epigastric pain. What is the most appropriate next step in the management of this patients illness? A. decreased appetite. Combination chemotherapy with cyclophosphamide. 5-fluorouracil. abdomen. she developed metastatic disease in the liver (five lesions in right and left lobe) and lungs (one lesion in the right and left lobe). After surgery. She was treated with irinotecan. along with mucosal thickening in the gastric antrum associated with a mass lesion. He has iron deficiency anemia. Approximately 20 months later. Results of upper gastrointestinal endoscopy reveal diffuse gastritis. welldifferentiated breast cancer. Sentinel node mapping and excision show that none of the three lymph nodes removed is positive for metastasis.

3-kg (5 Ib) weight loss. Poor performance status Hematology and Oncology:Question 86 A 57-year-old man has tried to stop smoking unsuccessfully for the past year. Daily isotretinoin. Daily dietary supplementation with the antioxidantl3-carotene B. Tamoxifen for 5 years D. Discontinuation of hydrochiorothiazide D. 50 mg/d orally. The patient takes aspirin. Bone scan shows multiple lesions. What is the most appropriate treatment for this patient’s hyponatremia? A.C. She has lost 11. increasing dyspnea on exertion. titrated to limit skin toxicity C. the ‘smokers cough” that he has had for years is a constant reminder that he is at risk of dying of lung cancer. Bronchoscopic biopsy specimen shows poorly differentiated adenocarcinoma. Which of the following findings would preclude use of palliative chemotherapy for this patient? A. A large lytic lesion is noted on the left femur. and fatigue. Daily bupropion D. Further staging studies suggest that the disease is limited-stage. distant breath sounds are audible in both lungs and there are scattered rhonchi. A solitary liver metastasis with normal serum bilirubin level B. Fluid restriction to 1 L/d and demeclocycline therapy C. what is the best recommendation for this patient? A. with erosion into the cortex. Abnormal laboratory results include hemoglobin of 12. Endobronchial biopsy reveals small-cell lung cancer. An annual low-dose spiral CT scan of the chest E. Hypercalcemia E. He smoked one pack of cigarettes per day for 49 years but quit smoking 2 years ago. Multiple liver metastases with a serum bilirubin level of 3. Radiation therapy to the left femur is initiated. A cytologically positive pleural effusion D. Combination chemotherapy for the small-cell lung cancer . and hydrochlorothiazide. Although he has no new symptoms. and CT scan of the chest shows a large left hilar mass and med iastinal lymphadenopathy. Fluid restriction to 1 L/d B. Review of systems is notable for intermittent cough.5 g/dL and a serum sodium of 127 meq/L. 81 mg/d. Chest radiograph shows a perihilar mass.3 kg (25 Ib) from baseline weight of 59 kg (130 Ib). He has tried smoking cessation counseling and use of nicotine gum. In addition to a smoking cessation program.0 mg/dL C. Tamoxifen and anastrozole for 5 years Hematology and Oncology:Question 85 A 59-year-old woman with an 80-pack-year smoking history is evaluated because of weight loss and severe pain in the upper part of her left leg. On physical examination. a 2. He wants to know what measures he can take that have been shown to reduce that risk. Sputum cytology every4 months Hematology and Oncology:Question 87 A 71-year-old man with mild chronic obstructive pulmonary disease and mild hypertension is evaluated during an annual routine visit.

What is the most appropriate management for this patient? A. Combination chemotherapy with bleomycin. but he notes little improvement in the swelling. His serum α-fetoprotein level is elevated at 100 ng/mL. Transvaginal ultrasound D. Her hair is growing back and her energy is returning. At the age of 32 years. Ciprofloxacin. Testicular ultrasound examination reveals a hypoechoic mass. She has just seen a television program on ovarian cancer. She also relates that she and her husband have been unable to have satisfying sexual relations because intercourse is painful for her. and she is taking tamoxifen. Inguinal orchiectomy Hematology and Oncology:Question 89 A 40-year-old woman has a routine gynecologic examination.Hematology and Oncology:Question 88 A 23-year-old man is evaluated because of a painless right-sided scrotal mass. and cisplatin C. Radiation to the pelvis D. Her periods cease while she is receiving chemotherapy. Her tumor was found to be rich in estrogen receptor. His digital rectal examination reveals a mildly enlarged prostate gland . A selective serotonin reuptake inhibitor and use of nonhormonal vaginal lubricating preparations B. except for mild hypertension. and she remains amenorrheic. but she is having severe hot flushes that keep her awake at night. Screening test for BRCA-1 and BRCA-2 Hematology and Oncology:Question 90 A 44-year-old woman has a lumpectomy and radiation therapy for stage II breast cancer. Rectovaginal pelvic examination in 1 year C. She has one older sister with hypertension and one younger brother who is healthy. Estrogen replacement therapy with a progesterone supplement D. Work-up for endometrial cancer because she is taking tamoxifen Hematology and Oncology:Question 91 A 64-year-old white man presents for serum prostate-specific antigen (PSA) screening at his wife’s urging. etoposide. and a paternal uncle was diagnosed with a type of lymphoma at 60 years. He is in generally good health. but she asks what additional tests she should have to look for ovarian cancer in the early stages that cannot be detected by physical examination. Her mother developed endometrial cancer at 56 years. and is concerned because previous use of fertility drugs and a family history of ovarian cancer were mentioned as possible risk factors. and his 13-human chorionic gonadotropin level is 64 m/UImL. She is so tired during the day that she is unable to perform her job in a satisfactory manner. What would be the most appropriate treatment for this patient? A. 500 mg every 12 hours. Blood test for CA-125 B. She receives 6 months of chemotherapy. she gave birth to twins after receiving fertility drugs. Results of her pelvic examination are unremarkable. is administered for 10 days. Retroperitoneal lymph node dissection B. Which of the following would be the most appropriate next step in his treatment? A. Reassurance that these normal physiologic responses to menopause will resolve in time C.

he has mild hepatomegaly. Four more cycles of chemotherapy. Exploratory laparotomy with resection of the primary tumor and placement of a hepatic arterial infusion pump D. and paracaval lymphadenopathy. . On physical examination. What is the most appropriate next step in the management of this patient? A. follow-up only Hematology and Oncology:Question 93 A 63-year-old man is evaluated because of a several-month history of increasing fatigue and some vague upper abdominal discomfort. and the findings suggest limited-stage disease.with no discrete nodules. His serum prostate-specific antigen (PSA) level was 7. Chest radiograph and CT scan show a right perihilar mass with mediastinal adenopathy. Colonoscopy reveals a nonobstructing. Best supportive care Hematology and Oncology:Question 94 A 69-year-old man underwent a radical prostatectomy ii years ago. including a taxane C. and his Gleason score was 5. His medical history includes gastroesophageal reflux disease. Fine-needle aspiration of one of the liver lesions confirms the presence of malignant cells consistent with a primary colon cancer. and serum aspartate aminotransferase level 65 U/L. What is the best recommendation concerning further therapy? A. After the patient completes mediastinal radiation therapy and four cycles of cisplatin and etoposide. clinical stage was Tic (clinically organ-confined and detected by screening PSA blood test only). bone scan. Because a digital rectal examination is more sensitive than a serum PSA test. His hemoglobin is 12. Combined regional chemotherapy to the liver and systemic chemotherapy C. CT scan of the abdomen and pelvis shows multiple hepatic lesions ranging in size from 1 cm to 4 cm. biopsy shows it to be poorly differentiated adenocarcinoma. Prophylactic cranial irradiation B. Resection of the localized disease D.1 ng/mL.2 g/dL. serum alkaline phosphatase level 280 U/L. The patient has been working full time. The results of CT scans of the abdomen. On the basis of his lack of symptoms it is unlikely that he has an elevated PSA D. mesenteric. non bleeding lesion. and a colonic mass at the splenic flexure. all of which are well controlled with medications. coronary artery disease. and MRI of the head are otherwise negative. No further therapy. What is the best advice to give him? A. There is insufficient evidence to establish whether PSA screening affects overall mortality rates C. There is no evidence that PSA screening leads to the earlier detection of prostate cancer B. repeat CT scans indicate that her disease is in complete remission. para-aortic. his normal examination makes it unlikely that he has cancer Hematology and Oncology:Question 92 A 58-year-old woman with a 60-pack-year smoking history is evaluated because of hemoptysis and weight loss. and clinical depression. He has no family history of prostate cancer. Examination of a specimen by bronchoscopic biopsy confirms small-cell lung cancer. Systemic chemotherapy E. Resection of the primary tumor followed by systemic chemotherapy B.

Hematology and Oncology:Question 95 A 61-year-old man with a 120-pack-year smoking history is evaluated for a persistent and worsening cough and found to have a 3-cm mass in the right upper lobe on chest radiography. What further treatment is appropriate for this patient? A. High-dose therapy with peripheral or bone marrow stem cell rescue E. 1 . bone scan. Increased risk of ovarian cancer . prolonged rise in his PSA. He remains asymptomatic.2 ng/mL. He is almost 70 years old. No further treatment beyond the proposed standard therapy B. Regional peritoneal therapy D. Her baseline risk of breast cancer qualifies her for consideration of tamoxifen (i. Four cycles of combination chemotherapy including a taxane B.His PSA level became detectable 4 years ago. She is interested in pursuing aggressive therapy and wishes to be treated as soon as possible in her local community. Her performance status is excellent. and 2. D. What is the most reasonable adjuvant therapy for this patient? A.4 ng/mL. Over the past 3 years. The patient undergoes a right upper lobectomy. Four cycles of combination chemotherapy including a taxane. and a recent bone scan revealed only degenerative disease. and pulmonary function tests indicate that the patient has adequate pulmonary reserve to undergo resection. The proposed systemic therapy will involve cisplatin or carboplatin and paclitaxel. The patients risk for rapid metastasis is low for which of the following reasons? A.66% over the next 5 years). He has had a slow. His PSA level became undetectable.e. No radiation therapy or chemotherapy is required at this time Hematology and Oncology:Question 96 A 47-year-old woman is recovering from surgery for stage III ovarian cancer (spread to the peritoneal cavity but without parenchymal liver involvement). the largest residual tumor was 3 cm in diameter. Decreased risk of myocardial infarction or ischemic heart disease B.. CT-directed needle biopsy is positive for squamous cell carcinoma. He was initially treated by radical prostatectomy. and all peribronchial lymph nodes are negative for tumor within the resected specimen. but her ovaries are intact. Six cycles of combination chemotherapy including a platinum agent E. B. Another drug that has a different mechanism of action C. followed by radiation therapy to the draining lymph node sites in the mediastinum C. All margins are clear. Radiation therapy to the draining lymph node sites in the mediastinum D. an absolute risk of at least 1. and MRI of the head are negative for metastatic disease. A second-look laparotomy Hematology and Oncology:Question 97 A 62-year-old postmenopausal woman with a family history of breast cancer in two first-degree relatives wishes to consider taking tamoxifen to reduce her risk of breast cancer. C. Positron emission tomography scan shows uptake only in the right upper lobe mass. Which of the following effects of tamoxifen are relevant to the decision? A. his PSA values have been 1. CT scans of the abdomen and pelvis. She has had a prior hysterectomy.8 ng/mL. Not all of the visible cancer could be resected. Increased risk of deep venous thrombosis C.

She owns a cat. The patients sister had breast cancer at 54 years and was treated by breast conservation therapy. She has no history of foreign travel. tamoxifen. and radiation therapy to the breast and axillary regions Hematology and Oncology:Question 100 A 32-year-old woman is evaluated because of a painless swelling in her lower neck. 4 years later she died of a recurrence of breast cancer. The lesion is completely excised. Chemotherapy and tamoxifen.0 ng/mL and the serum CA-125 is 1200 ng/mL (elevated). Combination chemotherapy B. It is firm but not rock-hard and is nontender. Chemotherapy and radiation therapy to the breast and axillary regions E. and she has been reading extensively on the Internet and has become confused about her options. The patient has seen two different surgeons with opposing viewpoints regarding the best treatment. She smokes but does not drink alcohol. Increased risk of major depression E. She has not had fever or unexplained weight loss. with no further treatment to the breast D. What is the best next step in this patient’s management? . Which of the following represents the best treatment for this patient? A. The serum carcinoembryonic antigen level is normal at 2. and pelvis shows mesenteric lymphadenopathy and a small amount of ascites but no ovarian masses. On physical examination. She noted the swelling about 3 weeks ago and now thinks it is getting larger. Radiation therapy to a wide port D. the tumor is found to be negative for hormone receptors. Modified radical mastectomy. Increased risk of colorectal cancer Hematology and Oncology:Question 98 A 50-year-old perimenopausal woman is evaluated because of abdominal swelling. She is otherwise healthy.5-cm moderately differentiated breast cancer. Bone scan to rule out skeletal metastases Hematology and Oncology:Question 99 A 50-year-old menstruating woman has a 1 . What is the best next step in her treatment? A. Debulking surgery C. Positron emission tomography scan to assess other sites of disease E. Chemotherapy. but has had night sweats twice in the past week. The remainder of her physical examination and results of a complete blood count are normal. She has been feeling fatigued lately and has noted dyspnea on exertion.D. she is alive and healthy at the age of 80 years. CT scan of the chest. and the margins of the excision are negative. Needle biopsy specimen shows adenocarcinoma. followed by tamoxifen and chemotherapy B. Chemotherapy with no further treatment to the breast C. Axillary node sampling shows that she has three positive nodes. abdomen. The tumor is negative for estrogen and progesterone receptors and is highly positive for HER2. a 3 X 5-cm left supraclavicular lymph node is palpable. Her mother had breast cancer at 62 years and was treated by mastectomy.

Perform a chest radiograph Hematology and Oncology:Question 101 A 64-year-old man is evaluated because of fatigue and shortness of breath on exertion. There are no significant findings on physical examination. and a lytic lesion is confirmed radiographically. and his serum creatinine level is 2. breast irradiation. D. and dullness to percussion. Observe for 2 weeks B. Her periods stopped during chemotherapy but resumed 4 months later. His serum calcium level is 11. A solitary liver metastasis is noted on CT scan and confirmed by needle biopsy and positron emission tomography scan. and decreased breath sounds in the base of .4 g/dL) with normochromic. Administer oral antibiotics and observe the course of the node D. A solitary brain metastasis is noted on CT scan of the head. and 3 months of chemotherapy. Physical examination shows palpable skin nodules over the affected breast. Chest radiograph shows a 4-cm right perihilar mass. A solitary bone metastasis is noted on bone scan. and bronchoscopic biopsy and cytologic evaluation of sputum confirm squamous cell carcinoma. Complete blood count is normal except for a serum alkaline phosphatase level at 110 U/L. Colon cancer E.9 mg/dL. and the patient reports no weight loss. Her original cancer was negative for estrogen and progesterone receptors and unequivocally positive for HER2. and the serum albumin level is low normal. and his stool is negative for occult blood on three measurements. he has pallor but is otherwise normal. Perform a needle aspiration of the node E. She is evaluated now because of pain in her back. a nagging cough.A. B. What is the most likely cause of his symptoms? A. and cytopathologic examination of the pleural fluid is positive for tumor. Small-cell lung cancer Hematology and Oncology:Question 102 A 63-year-old man is evaluated because of new-onset hemoptysis and a 100-packyear smoking history. Results of a positron emission tomography scan are negative.8 mg/dL. axillary adenopathy. In which of the following situations would surgical therapy be offered? A. C. Hematology and Oncology:Question 103 A 40-year-old woman was treated for breast cancer 5 years ago by lumpectomy. He denies blood loss. except for the right perihilar mass. Multiple myeloma B. Refer her to an otolaryngologist for a thorough search for a primary lesion of the oropharyngeal mucosa C. Chronic pyelonephritis C. He is anemic (hemoglobin 8. He has had three episodes of urinary tract infection in the past 7 months. and fatigue. A small effusion is noted in the right hemithorax. On physical examination. The total serum protein level is elevated. Iron deficiency anemia D. CT scans of the chest and abdomen show no mediastinal adenopathy. and she remains premenopausal now. normocytic indices.

One year ago. Bone scan and MRI are consistent with bone metastases. the Gleason score will not be predictive of outcome. and his serum prostatespecific antigen (PSA) level is found to be 6. but no discrete nodules. The patient reports a 13. Trastuzumab and taxane-based chemotherapy E.7 ng/mL. it is negative for estrogen and progesterone receptors. The Gleason score indicates that the patient would have a high risk of recurrence after a radical prostatectomy. Hospice care and comfort measures B. loss of appetite. Biopsy of one of the skin lesions is consistent with metastatic breast cancer. The Gleason score is not reproducible and should not be used as a prognostic variable. The patient is referred to a urologist who performs a biopsy of the gland. and positive for HER2. Digital rectal examination reveals a mildly enlarged prostate gland. Diagnostic CT-guided biopsy under a heparin window” when the patients clinical . CT-guided biopsy of the hepatic lesions and the pancreatic mass is deferred during this hospitalization.6 g/dL Serum alanine aminotransferase 65 U/L Serum aspartate aminotransferase 78 U/L CA 19-94500 U/L Arterial oxygen saturation 85% by pulse oximetry Spiral CT scan of the chest shows a pulmonary embolism. Hematology and OnCology:Question 105 A 78-year-old man is evaluated because of shortness of breath of acute onset. Combination endocrine therapy with ovarian ablation and an aromatase inhibitor Hematology and Oncology:Question 104 A 65-year-old man has a routine screening examination. Even before the recent episode of dyspnea. his PSA was 2. Subsequent CT of the abdomen shows a 4-cm mass in the tail of the pancreas and numerous low-attenuation lesions in the liver. her liver edge is palpable below the right costochondral border. Radiograph of the chest shows multiple pulmonary nodules and a left pleural effusion confirmed by CT. Best supportive care and referral to a hospice B. his physical activity has been limited. He has hypertension and symptomatic atherosclerotic coronary artery disease despite optimal medical management. Which of the following best describes his prognosis on the basis of his Gleason score? A. Combination endocrine therapy with tamoxifen and an aromatase inhibitor C. Laboratory studies: Hemoglobin 10. B. he spends most of the day resting either in bed or in a chair.6-kg (30-Ib) weight loss. C.1 ng/mL. The Gleason score indicates that the patient would have a very low risk of recurrence after radiation therapy.the left lung. nausea. Liver function values are approximately twice normal. Because the patient is asymptomatic. vague upper abdominal discomfort. Biopsy specimen shows adenocarcinoma of the prostate with a Gleason score of 9. serum bilirubin is normal. High-dose chemotherapy with bone marrow stem cell support D. and fatigue.5 g/dL Serum albumin 2. What is the most appropriate treatment for this patient? A. D. What is the most appropriate next step in the management of this patient? A. The patient is hospitalized for anticoagulation and oxygen therapy and gradually improves. which also shows the liver nodules.

Chemotherapy followed by radiation therapy Hematology and Oncology:Question 108 A 78-year-old woman with metastatic breast cancer involving the bones and soft tissues who has been taking hormone replacement therapy is evaluated in the emergency department because of lethargy and weakness.4 mg/dL Serum creatinine 1. Intravenous administration of a bisphosphonate C.status improves C. Bone thinning C. Bone scan and CT scan of the head are negative for tumor. Surgery followed by chemotherapy B. Which of the following is a potential side effect of leuprolide that the patient should be informed about before commencing therapy? A. She has a history of congestive heart failure that has been controlled with medications. Radiation therapy and concomitant chemotherapy E. Radiograph of the chest shows a right hilar mass and mediastinal widening. nausea. Neurolytic celiac axis block D. Palliative radiation therapy Hematology and Oncology:Question 106 A 76-year-old man was diagnosed with prostate cancer 8 years ago. and a clinical stage of T2b (organ-confined on digital rectal examination but involving both lobes of the prostate gland). Palliative chemotherapy E. A low serum sodium level and inappropriately high urine osmolality suggest that he has the syndrome of inappropriate antidiuretic hormone secretion. Slow rehydration with half-normal saline B. Bronchoscopic biopsy specimen shows small-cell lung cancer.1 ng/mL. a Gleason score of 6. thirst.4 ng/mL. his PSA level began to rise and now is 14. Radiation therapy followed by chemotherapy C. and dizziness. CT scans of the chest and abdomen confirm the hilar mass and bulky lymphadenopathy in the mediastinum with no other overt metastases. Weight loss D. His urologist has suggested initiating a course of leuprolide. Four years later.6 mg/dL Serum albumin 3. He was treated with external-beam radiation therapy. Chemotherapy alone D. Increased hair growth Hematology and Oncology:Question 107 A 68-year-old man with a 60-pack-year smoking history is evaluated because of hemoptysis. Vigorous rehydration with normal saline . She is orthostatic and clinically dehydrated. At that time he had a serum prostate-specific antigen (PSA) level of 12 ng/mL. Which of the following is the most appropriate treatment recommendation? A. and his PSA level dropped to a low of 1. Impaired urinary flow B. A recent bone scan and CT scans revealed no metastatic disease.0 g/dL What is the most appropriate initial treatment? A. Laboratory studies: Blood urea nitrogen 42 mg/dL Total serum calcium 11 .

She is concerned that “cancer runs in the family” and would like to have one of those “gene tests” she has read about. who was a smoker. cytokeratin. Biopsy specimen shows a poorly differentiated malignancy that is difficult to characterize by light microscopy. Bone scan B. Intravenous administration of corticosteroids Hematology and Oncology:Question 109 A 52-year-old woman who is a nonsmoker has a 3-cm right inguinal lymph node. Treatment included orchiectomy and retroperitoneal lymph node dissection. Radiation therapy to the chest D. Her father had prostate cancer at 82 years of age. Combination chemotherapy with bleomycin.61 to 3. Intravenous administration of furosemide along with saline rehydration E. A chest radiograph shows a widened superior mediastinum. who have no history of cancer. The biopsy specimen is negative for leukocyte common antigen. He now presents with an elevated level of β-human chorionic gonadotropin hormone and multiple pulmonary lesions ranging in size from 0. . What is the most appropriate initial intervention for this patient? A. He is asymptomatic. and a sensation of head fullness. Surgical resection of the lung lesions B. Stain for S-100 D. and 65 years who have no history of cancer. now aged 58. What is the best treatment for this patient? A. and her mother had breast cancer at age 71 years. Mediastinoscopy with biopsy E. Physical examination is notable for cervical venous distention and facial edema.D. Measurement of serum carcinoembryonic antigen C. Watchful waiting Hematology and Oncology:Question 112 A 64-year-old woman has the following family history: One sister. etoposide. spread from the primary tumor to retroperitoneal lymph nodes) nonseminomatous germ cell tumor 1 year ago. CT of the chest and a venous dye study to rule out thrombosis of the superior vena cava D.22 cm. had lung cancer at the age of 63 years. 60. She has two daughters and two sons between the ages of 25 and 39 years. and cisplatin C. Her ancestry is European in general. Ventilation/perfusion scan Hematology and Oncology:Question 111 A 29-year-old man was diagnosed with stage II (that is. Needle biopsy specimen of the largest lesion reveals a germ cell tumor consistent with the initial diagnosis. Which of the following additional tests would best establish the source of this tumor? A. Measurement of serum CA 19-9 Hematology and Oncology:Question 110 A 59-year-old man who is a heavy smoker presents with cough. and she is not of known Ashkenazi Jewish descent. CT of the chest and consultation with a pulmonologist and a thoracic surgeon C. shortness of breath. She has three other sisters. and estrogen receptors. Initiation of radiation therapy while a diagnostic work-up is pursued B. another sister had breast cancer at the age of 70 years.

What is the best advice to give her and her family? A. Have blood testing for abnormalities in known germ-line tumor suppressor genes (such as BRCA-1 and BRCA-2). B. Encourage her children to have genetic counseling and testing for abnormalities in known germ-line tumor suppressor genes. C. Advise her children to start routine cancer screening tests immediately, including mammography, colonoscopy or sigmoidoscopy, and prostate-specific antigen testing. D. Advise her that genetic testing is unnecessary, and recommend that she and her family continue screening and risk-reduction strategies as recommended for the general population. E. Recommend bilateral prophylactic mastectomies and oophorectomies for her and her daughters. Hematology and Oncology:Question 113 A 72-year-old man is evaluated because of back pain and fatigue. Laboratory values indicate he is anemic, with normal leukocyte count and platelet count. He has mild hypercalcemia and a normal serum creatinine level. His gamma globulins are elevated, and serum protein electrophoresis shows the presence of a monoclonal protein, an IgG-K light chain containing immunoglobulin at 4.4 g/dL. His bone marrow contains 20% plasma cells. A skeletal survey reveals multiple osteolytic lesions in the spine, ribs, and skull. Therapy with melphalan and prednisone is initiated. Which of the following agents would NOT be routinely used in his management? A. Erythropoietin B. Radiation therapy C. Intravenous gamma globulin D. Adequate analgesia (including narcotics, if necessary) E. A bisphosphonate

ANSWERS Hematology and Oncology:Question 1 The correct answer is D Educational Objectives Diagnose thrombocytopenia in a critically ill patient. Critique The acute onset of a febrile illness accompanied by leukocytosis, hypotension, and thrombocytopenia is consistent with sepsis, although the cause may not be immediately evident. Routine studies not mentioned should involve blood and urine cultures, and chest radiography with sputum examination and culture if indicated. As always, the peripheral blood smear should be examined closely in any patient with hematologic abnormalities. The levels of fibrin D-dimer and total fibrinogen should be assessed in this patient, in conjunction with routine coagulation studies. Increased levels of D-dimer and lownormal or decreased levels of fibrinogen would be consistent with disseminated intravascular coagulation.

Since the patient was known to be in previously good health without underlying hematologic disease, it is likely that the thrombocytopenia is acute and results from accelerated platelet clearance. Therefore, a bone marrow aspiration and biopsy are not likely to be helpful. Measuring factor VIII will not provide useful information in this case—the level may be normal, decreased due to consumption, or increased due to acute inflammation. Platelet-associated IgG levels are elevated in many disorders, including sepsis, and therefore will not provide useful information concerning the likelihood of a primary immune-mediated thrombocytopenia. The bleeding time has poor predictive value for bleeding and is usually prolonged in thrombocytopenic patients. Diagnosis of disseminated intravascular coagulation would provide a likely explanation for the patients thrombocytopenia, although sepsis alone may lead to decreased platelet counts. Moreover, the coagulation status in a thrombocytopenic patient should be assessed, and factor replacement initiated if indicated. Hematology and Oncology:Question 2 The correct answer is E Educational Objectives Treat a first unprovoked episode of pulmonary emboli provoked by a transient risk factor. Critique The duration of the anticoagulation therapy this patient has received is appropriate (3 to 6 months) for a first episode of venous thromboembolism. An episode of pulmonary embolism that is associated with a transient risk factor (such as oral contraceptive use) carries a substantially lower annual risk of recurrence than one that is “unprovoked.’ Although heterozygosity for the prothrombin G2021 0A mutation is a risk factor for a first episode of unprovoked embolism (and embolism in association with oral contraceptive use), its presence does not confer an increased risk of recurrence. She should not resume taking oral contraceptives and be made aware of the symptoms of recurrent thromboembolism. The recurrence-prevention benefits of anticoagulation at an INR of 2 to 3 for more than 3 to 6 months have not been demonstrated to outweigh the bleeding risk associated with extended treatment. Although low-intensity warfarin (target INR 1 .5 to 2) was recently shown to protect against recurrent venous thrombosis without a significant increase in bleeding risk, this patient’s relatively low annual recurrence risk in the absence of provocative risk factors would argue against the continued use of even low-intensity warfarin. The extent to which oral anticoagulation at a target INR of 1.5 to 2 provides antithrombotic protection to women with a history of pulmonary embolism and continued use of oral contraceptives is unknown; it is not a preferred recommendation for this patient. Aspirin or a combination of aspirin and dipyridamole has not been shown to decrease the risk of recurrent venous thromboembolism, although aspirin is effective in the primary and secondary prevention of myocardial infarction. Hematology and Oncology:Question 3 The correct answer is B Educational Objectives Recognize therapeutic options in patients with sickle cell disease. Critique Hydroxyurea therapy reduces the incidence of sickle-cell-related events through its capacity to re-activate expression of the developmentally silenced fetal gamma globin. The potential toxicities of hydroxyurea are well described. The patients levels can be easily monitored through periodic complete blood counts. The patient’s

current transfusion-related decrease in hemoglobin S should not affect the decision to initiate therapy, because the benefits of hydroxyurea are generally delayed. Although bone marrow transplantation might be a consideration for this patient, it is attended by substantially higher toxicity than hydroxyurea therapy. Gene therapy approaches for the treatment of sickle cell disease remain investigational. There is no role for either long-term transfusion therapy or oxygen therapy for this patient. Hematology and Oncology:Question 4 The correct answer is B Educational Objectives Diagnose and treat drug-induced neutropenia. Critique The patient should be treated with granulocyte colony-stimulating factor. Two types of neutropenia are reported with clozapine. One is mild-to-moderate neutropenia (absolute neutrophil count, 500 to 1500 μ/L) that occurs in 1.5% to 2.0% of patients receiving clozapine. When clozapine is discontinued, recovery occurs within 3 to 7 days, and patients remain asymptomatic. The second type of neutropenia is more severe. It consists of agranulocytosis (absolute neutrophil count less than 500/μL) and has an incidence of 0.8% in patients treated for 1 year. Most cases of agranulocytosis occur within 3 months after the start of treatment. All affected patients are at risk for neutropenic sepsis. There is usually a complete, selective cessation in the bone marrow of neutrophil-precursor production beyond the myeloblast stage. Recovery requires 14 to 22 days; G-CSF therapy has been shown to shorten this duration. Given the patient’s history of clozapine use and the absence of anemia and thrombocytopenia, it is not necessary to perform bone marrow aspiration and biopsy unless her neutropenia fails to improve over the next 2 weeks. Granulocyte transfusions are reserved for patients with uncontrolled infection despite antibiotic therapy and persistent severe neutropenia. Drug-induced neutropenia commonly resolves after discontinuation of the drug, and bone marrow transplantation is not warranted. This patient reported no HIV risk factors, and her presentation does not suggest HIV infection.

Hematology and Oncology:Question 5 The correct answer is A Educational Objectives Understand the differential diagnosis of anemia in severe hepatic failure. Critique The patient has spur cell anemia. In a small percentage of patients with alcoholic cirrhosis, erythrocytes accumulate large amounts of free cholesterol with significant erythrocyte membrane distortion. A progressive severe hemolytic anemia ensues, with cells that partly resemble acanthocytes. Unlike true acanthocytes, the cells in spur cell anemia are small and spheroidal with short, spiny projections (hence the term spur cell). Increased amounts of membrane cholesterol lead to increased membrane surface area. Membrane distortion forms the basis of the morphologic presentation secondary to abetalipoproteinemia. The spur cells are trapped and destroyed in the splenic cords leading to progressive splenic enlargement. The prognosis of patients with spur cell anemia is extremely poor. Splenectomy is

The clinical picture is characteristic of anemia of chronic disease. but it would not account for her severe anemia and elevated reticulocyte count. may be associated with membrane changes such as target cell formation. which complicate the procedure. With an increase in cholesterol.occasionally effective in moderating the hemolysis. but his reticuloendothelial iron stores are adequate. hypersplenism. Hematology and Oncology:Question 7 The correct answer is E Educational Objectives Recognize the presentation of delayed hemolytic transfusion reaction. as can happen. If the antibody titer decays to a subdetectable level. and the cell membrane surface area increases. hemoglobin collects in the thicker areas of the cells (the center and edges) producing a “target’ appearance. which often includes mild microcytosis. it would be appropriate to focus treatment on the osteomyelitis alone. alcohol as a direct toxin to erythroid progenitor cells). Hematology and Oncology:Question 6 The correct answer is D Educational Objectives Treat symptomatic anemia of chronic disease. however. Multitransfused and multiparous recipients are at risk for prior erythrocyte alloimmunization. patients with alcoholic liver disease may have chronic. the cholesterol : phospholipid ratio changes. Thus. the cells become broad and flat. Liver disease. mild to moderate anemia that is multifactorial (liver disease. but patients usually have underlying portal hypertension and coagulopathies. His serum iron and total iron-binding capacity are low. As the cells dry. if the alloantibody is specific for a low-frequency antigen not represented on the reagent cells in the antibody screen. the high reticulocyte count makes this diagnosis unlikely. The time course and presentation are typical for a delayed hemolytic transfusion reaction arising from an anamnestic response to rechallenge by erythrocyte antigens after previous sensitization. If the patient were asymptomatic. pretransfusion testing will not detect the . for which erythropoietin (usually given concurrently with iron) is an effective therapy. particularly extrahepatic biliary obstruction. Because the anemia is associated with symptoms. These antibodies are usually detected in the pretransfusion antibody screen by means of reagent cells that carry most clinically significant antigens. Conversely. Folic acid deficiency should be considered in any patient with alcoholism who has a macrocytic anemia. specific therapy for anemia is indicated. Critique The patient is having a delayed hemolytic transfusion reaction. Cyanocobalamin is a treatment for vitamin B12 deficiency but not for anemia of chronic disease. More often. The patient probably has preexisting hypersplenism. Iron therapy by itself is not effective in anemia of chronic disease. In this patient. as demonstrated by his elevated serum ferritin level. Critique The patient has anemia of chronic disease. based on her diagnosis of cirrhosis. the antibody screen or even full crossmatching will be negative using a pretransfusion sample. Patients who have chronic alcoholism should be maintained prophylactically on folic acid supplementation.

but the fact that this patients direct antiglobulin test was negative makes it unlikely that her symptoms are attributable to autoimmune hemolysis. A bone marrow aspiration and biopsy should be performed to confirm the diagnosis. There is usually no problem because most donors are negative for low-frequency antigens. but not detected. radiation therapy. Glucose-6-phosphate dehydrogenase deficiency is Xlinked and rare in females. If the diagnosis of HFE hemochromatosis is made before the development of cirrhosis. It causes immediate rather than delayed hemolysis in response to oxidant stress. Given his history of chemotherapy. Failure to keep a unit fully refrigerated can cause erythrocytes to become nonviable. . In general. in a C282Y homozygote. Critique Diagnostic liver biopsy is the procedure of choice. This situation causes a more acute presentation of the hemolysis. A finding of low amounts of iron in the usual storage sites of bone marrow macrophages is a characteristic of HFE hemochromatosis. there is an increased risk for the development of hepatocellular carcinoma even after removal of excess body iron. because preformed. If the diagnosis of hemochromatosis is made after the development of cirrhosis. along with a therapeutic phlebotomy program. however. Occasionally. and does not cause a positive result on a direct antiglobulin test. the current finding of pancytopenia strongly suggests that he has secondary myelodysplastic syndrome or leukemia. a diagnostic liver biopsy should be recommended if the serum ferritin concentration is greater than 1000 ng/mL or if liver function test results are elevated at the time of diagnosis. The effects of transfusion with such a unit are seen within 24 hours as the cell stroma and supernatant hemoglobin are cleared and the nonviable cells are destroyed. Although the diagnosis of hereditary hemochromatosis can be made on the basis of homozygosity for the C282Y mutation in the HFE gene.incompatibility unless a full crossmatch is performed. then therapeutic phlebotomy to remove excess iron stores and to prevent reaccumulation should enable the patient to have a normal life expectancy. Swelling of the first and second metacarpophalangeal joints is a characteristic but not necessary finding in the diagnosis of HFE hemochromatosis. It is important to proceed with appropriate diagnostic and therapeutic measures. important prognostic information can be obtained from a diagnostic liver biopsy. Critique This patient presents with pancytopenia and infection. and anemia. most likely pneumonia. hemolysis occurs during storage from excessive red blood cell metabolic activity. Errors in ABC compatibility show up immediately rather than after 7 days. Hematology and Oncology:Question 9 The correct answer is D Educational Objectives Diagnose myelodysplastic syndrome secondary to chemotherapy and radiation therapy. antibody is present. a patient might receive a low-frequency antigen-positive unit. Hematology and Oncology:Question 8 The correct answer is D Educational Objectives Diagnose hereditary hemochromatosis. treatment of hemochromatosis with iron removal can arrest organ damage and improve survival. Transfusion can trigger autoimmune hemolysis.

Critique No testing is necessary. but it is not advisable to test for these abnormalities upon presentation with an acute thrombotic event or after the initiation of anticoagulant therapy. It is possible that she has a deficiency of protein C. Critique The sole reason for blood irradiation is to prevent transfusion-related graft-versushost disease (GVHD) in patients at risk for it. Gamma irradiation of cellular blood products prevents transfusion-related GVHD by rendering donor T cells nonviable and incapable of mounting a GVHD response. Hypothyroidism can result in anemia but rarely pancytopenia. Hematology and Oncology:Question 11 The correct answer is E Educational Objectives Select the appropriate timing for a work-up for a hereditary hypercoagulable state. alloimmune sensitization. and gastrointestinal system under immunologic attack. It is best to wait at least 2 weeks after anticoagulants are discontinued (upon completion of 3 to 6 months of therapy) to test for these deficiency states. protein 5. or antithrombin III. Transfusion-related GVHD has a high mortality rate. skin. Higher doses may interfere with the function or survival of red cells and platelets. the patient should be started on anticoagulation therapy.This lethal late complication is seen in 5% of Hodgkins disease survivors treated with multimodality therapy. This patients clinical presentation is not consistent with severe sepsis and associated pancytopenia. Testing for a lupus . Irradiation at this dose has no effect on the transmission of cytomegalovirus (or any pathogen). or leukocyte antigenicity. Relapse of Hodgkins disease with bone marrow infiltration and pancytopenia is uncommon and very unlikely 8 years after successful therapy. Not only are the liver. Although the cells are nonviable and will not cause a sustained attack against the host. Most of the myelodysplastic syndromes and acute leukemias occur 3 to 11 years after therapy. it is unlikely that she has the factor V Leiden or prothrombin G2021 0A mutation because these abnormalities are not found in native Asian populations. Therefore. Irradiation has no effect on any of the mechanisms of transfusion-induced hemolysis. Hematology and Oncology:Question 10 The correct answer is C Educational Objectives Recall the purpose of blood irradiation prior to transfusion. The dose used is between 25 and 50 Gy. Although her family history suggests that she has an underlying hereditary defect predisposing to venous thromboembolism. There is no history of new drug intake to suggest drug-associated bone marrow suppression. Leukocyte reduction is not sufficient for this purpose because it leaves behind too many cells. but the recipients bone marrow is also destroyed by the donor T cells. Deletions or loss of chromosomes 5 and 7 is commonly associated with alkylating agent therapy. they can still elaborate cytokine. irradiation will not prevent febrile reactions.

The yield of a search for an occult malignancy in a young woman without any suggestive symptoms or signs would be extremely low. Hematology and Oncology:Question 12 The correct answer is D Educational Objectives Recognize the clinical presentation and laboratory findings of paroxysmal nocturnal hemoglobinuria Critique Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon acquired chronic disorder of hematopoietic stem cells. but it should not be done in the presence of heparin. Even if clotting factor levels were adequate for hemostasis before transfusion. Even if a sample drawn for another purpose was found. A history of dark-colored urine indicative of hemoglobinuria is noted in only 25% of patients. the blood bank would be unable to use it to determine .anticoagulant is reasonable at presentation. Use of fresh frozen plasma avoids the risk of causing polycythemia. furthermore. This defect leads to absence of certain membrane proteins in affected hematopoietic stem cells. pancytopenia. There is marked reticulocytosis. and high serum lactate dehydrogenase levels. and moderate to severe thrombocytopenia. In PNH. An acquired somatic mutation in the PIGA gene results in failure to express phosphatidylinositol-glycan anchor. The best choice for clotting factor replacement in this circumstance is group AB fresh frozen plasma. leukopenia. therefore. antiphospholipid antibody syndrome). laboratory evaluation typically shows severe anemia. A diagnosis of autoimmune hemolytic anemia would not explain the leukopenia and thrombocytopenia. In an emergency when a patient’s ABC type is unknown. her clinical history is more suggestive of a hereditary than an acquired thrombotic disorder (that is. It is characterized by attacks of intravascular hemolysis and hemoglobinuria. Recurrent abdominal pain is common in PNH and is probably related to portal or mesenteric vein thrombosis. Aplastic anemia is not associated with hemolysis or splenomegaly. Hemoglobin in the urine is reabsorbed by the proximal convoluted tubules and excreted as hemosiderin. Myelodysplastic syndrome and acute myeloid leukemia are not associated with severe hemolysis as seen in this case and are associated with characteristic peripheral blood smear findings. There is evidence of intravascular hemolysis with low or absent haptoglobin. Hematology and Oncology:Question 13 The correct answer is D Educational Objectives Recall the principles of ABC compatibility Critique This patients liver disease makes it very likely that his ability to synthesize clotting factor is decreased. although the absolute reticulocyte count is often relatively low for the given degree of anemia. and the finding helps differentiate this disorder from aplastic anemia. and recurrent venous thromboses. ABC hemagglutinins arise naturally and are therfore expected to be present in plasma. Moderate splenomegaly is common. replacement therapy is warranted. and targets erythrocytes for complement-mediated destruction. the levels would have been diluted through replacement of his blood volume. Group O packed cells have neither A nor B antigens and can be used since they are the “universal donor” and are compatible with all patients’ plasma. including proteins that regulate complement activation. Diagnosis can be made by showing erythrocyte sensitivity to acid-induced hemolysis (Hams test) or lack of CD55 and CD59 proteins on erythrocytes demonstrated by flow cytometry.

The erythrocyte P-antigen is the receptor for the parvovirus. thrombocytopenia. It is considered safer to use unmatched Group O packed cells rather than risk making a mistake leading to ABC incompatibility. blood. human parvovirus is able to infect patients with a particular tropism for erythroid precursors. and most patients do not require treatment. The bone . The infection produces large. Individuals who are group AB have both A and B antigens on their red cells. HIV infection can produce nonspecific changes sometimes associated with dysplasia. Direct antiglobulin tests are positive in 20% of patients with HIV. Just as group O is the universal donor for red cells. Drug-induced glucose-6-phosphate dehydrogenase deficiency and hemolysis represent a possible but unlikely diagnosis in a person of northern European extraction.S. Food and Drug Administration requirements). Either an intrinsic defect of the stem cells or immune-mediated stem cell destruction leads to transfusion-dependent anemia. Because of the large amount of donor plasma in whole blood as opposed to packed red cells. group AB is the universal donor for plasma. which is usually mild. but not distinct from other viral infections. and so are incapable of making naturally occurring anti-A or anti-B. The relatively unremarkable peripheral blood smear and the “classic” marrow findings make glucose-6-phosphate dehydrogenase hemolysis very improbable. it must be group specific. Intravenous immunoglobulin contains antiparvoviral IgG and neutralizes the infection with resolution of the anemia. Whole blood is a poor choice to treat coagulopathies because it loses labile factors during storage. but this condition of autoimmunity rarely produces serious anemia. a rapidly fatal disorder in which myeloid progenitor and stem cells in the bone marrow are severely diminished or absent. This patient’s rapidly progressive anemia and reticulocytopenia would be inconsistent with marrow suppression from use of trimethoprimsulfamethoxazole. leading to a complete cessation of erythropoiesis with associated reticulocytopenia. and severe neutropenia. and so group O whole blood can only be given safely to group O recipients. or marrow with the use of polymerase chain reaction amplification of DNA. Against a background of underlying immune deficiency. Drug-induced marrow suppression is common in patients with HIV and is often related to antiretroviral agents as well as drugs such as trimethoprimsulfamethoxazole.ABC type unless it were proven to be properly labled and the recipient’s identity had been positively confirmed at the time of draw (U. The diagnosis is confirmed by the demonstration of parvovirus in the serum. Critique Human parvovirus B19 can cause severe chronic anemia in patients with HIV infection. Hematology and Oncology:Question 14 The correct answer is B Educational Objectives Diagnose progressive anemia in a patient with HIV infection. HIV in general suppresses erythropoiesis and produces a mild anemia that sometimes responds to erythropoietin therapy. Hematology and Oncology:Question 15 The correct answer is B Educational Objectives Diagnose and treat severe aplastic anemia. Critique This patient has aplastic anemia. multinucleated dysplastic proerythroblasts. Infection produces intracellular products that are directly toxic to erythroid precursors.

It is common to see relative abundance of plasma cells in severely hypoplastic bone marrow of patients with aplastic anemia. low platelet counts are rarely a cause of bleeding. Platelet concentrates would not be appropriate for this patient. Melphalan and prednisone are effective in multiple myeloma. but in this application it is used chiefly as a patch for serosal or other surface tears rather than to stop diffuse bleeding. and a few other cold-insoluble proteins. it is often a sign of disseminated intravascular coagulation or tissue ischemia causing a superimposed consumptive coagulopathy. Cryoprecipitate is devoid of most coagulation factors and serves as a concentrated source of fibrinogen. Long-term transfusion therapy will not correct the severe neutropenia and risk of life-threatening infections. Intravenous immunoglobulin is useful in pure red cell aplasia due to parvovirus B19 infection. myelodysplastic syndromes. Cryoprecipitate can also be used as a fibrinogen source in the making of fibrin glue used as a biologic sealant. as in this case. Hematology and Oncology:Question 16 The correct answer is C Educational Objectives Recognize dilutional coagulopathy as a consequence of massive transfusion. making this option less attractive for young patients. Clinical bleeding during massive transfusion is almost always the consequence of depletion of clotting factors through blood loss. and it does not affect leukocyte and platelet counts. Parvovirus infection does not cause severe anemia in healthy individuals who can mount an antibody response. Immunosuppression with antithymocyte globulin and cyclosporine can induce responses but is usually reserved for patients who do not have a suitable donor for allogeneic stem cell transplantation or who are too old for this procedure. Allogeneic stem cell transplantation is the treatment of choice. Whole blood (which is almost never available because it is needed for component manufacture) would not help with hemostasis. severe thrombocytopenia can result. After more than three blood volumes have been replaced. Critique This patient most likely has dilutional coagulopathy as a result of the massive transfusion with packed red blood cells and crystalloid. It can also be a function of dilution by intravenous fluid and banked blood units that lack clotting factors. . especially when a dilutional problem is involved. Its major use is as an adjunct to fresh frozen plasma when additional fibrinogen is required.marrow is typically aplastic or hypoplastic. particularly for patients younger than 50 years with HLA-identical sibling donors. Transplantation can result in long-term survival of 75% to 90%. The development of more purified products to treat hemophilia and von Willebrand’s disease has obviated the use of cryoprecipitate as a stand-alone agent to treat bleeding. without evidence of infiltrating malignant cells. Long-term follow-up of patients treated with immunosuppressive therapy shows an increased incidence of myelodysplastic syndrome and leukemia. and the platelets become nonviable rapidly during refrigerated storage. and drug-induced or nutritional cytopenias. The best therapy is administration of fresh frozen plasma to aid in restoration of normal coagulation ability. The plasma in whole blood loses labile clotting factors during storage. When severe thrombocytopenia occurs earlier. factor VIII. The differential diagnosis includes acute leukemia (particularly hypoplastic leukemia). This patient does not have a plasma cell disorder. however.

Critique It is never possible to guarantee that any given patient will not experience a bleeding complication. Therefore. dental procedures. in this regard it should not be considered superior to the bleeding time. it has not. probably because it is attributed to the underlying malignancy or its treatment. A thorough patient history. It cannot. They can. it is unlikely that the patient will benefit from further therapy. be normal in von Willebrand’s disease or in moderate deficiencies of factors VIII or IX that are sufficient for normal hemostasis but may nonetheless predispose a patient to surgical bleeding.Hematology and Oncology:Question 17 The correct answer is C Educational Objectives Diagnose and treat chemotherapy-induced anemia and fatigue. therapy with erythopoietin and longer-acting darbepoetin has been shown to decrease transfusion requirements. Hematology and Oncology:Question 18 The correct answer is D Educational Objectives Understand the use of platelet function tests as screening tests for surgical bleeding. and if there is no response by 6 weeks. been validated as a screening test for surgical bleeding in unselected patients. however. These studies are expensive and have not been established as preoperative screening tests. these episodes reflect surgical lesions rather than an underlying predisposition to bleeding. Administration of androgens plays no proven role in the treatment of fatigue associated with chemotherapy-induced anemia. Critique Weekly administration of erythropoietin would be appropriate for this patient. however. Platelet aggregation studies should be done only after a screening test such as the bleeding time or the PFA-i 00 analysis suggests an underlying platelet disorder. focusing particularly on previous surgery. The prothrombin time and partial thromboplastin time may be prolonged in hemophilia A or B or in deficiencies of other intrinsic pathway factors such as factor XI or factor XII. Response to erythropoietin therapy usually occurs within 3 to 5 weeks. it is not recommended because of the risk that the cancer would progress. In most of these patients. remains the best method of detecting a patient who may be at risk for increased bleeding during surgery. increase hemoglobin levels. Granulocyte colony-stimulating factor stimulates granulocytic precursors in the bone marrow and does not affect hemoglobin levels. Although cessation of chemotherapy would eventually allow recovery from anemia. and improve quality of life. Vitamin E supplements have not been shown to improve anemia and fatigue related to cancer chemotherapy. however. however. The bleeding time might be a useful test when screening for inherited or acquired deficiencies in platelet function in a patient with an appropriate history. menstrual bleeding. the partial thromboplastin time is not a useful presurgical screening test (although it has been suggested that the partial thromboplastin time be used to screen patients of Ashkenazi Jewish descent because of the high incidence of factor Xl deficiency in this . predict surgical bleeding when used to screen unselected surgical patients. Therefore. The Platelet Function Analyzer-100 (PFA-100) may be more sensitive and perhaps more specific in the diagnosis of platelet disorders and von Willebrand’s disease than bleeding time. Compared with placebo. Approximately 75% of patients being treated for cancer experience fatigue. In most cases. and bleeding with prior trauma. the fatigue is not treated.

an open lung biopsy or a transbronchial biopsy would be appropriate next steps if the marrow biopsy did not reveal a . liver biopsy probably would yield a diagnosis but has a higher procedure risk profile in an elderly patient compared with bone marrow biopsy. anemia. who have been exposed to corticosteroids or other immunosuppressive agents. Hematology and Oncology:Question 20 The correct answer is B Educational Objectives Diagnose leukoerythroblastosis due to tuberculosis. Tuberculosis is increasingly recognized in elderly patients who lack pulmonary symptoms. Cultures of the marrow would likely grow acid-fast bacilli. Nearly 90% of patients with miliary tuberculosis have anemia. The history and examination findings largely rule out noncompliance with treatment. In this patient. myelofibrosis with myeloid metaplasia. the physician should order a serum ferritin test to correctly characterize the anemia syndrome. and the anergy noted by skin testing should raise a very high degree of suspicion of disseminated tuberculosis. At the very least. In the presence of abnormal liver function studies. Furthermore. and whose findings slowly evolve toward a picture consistent with an infiltrative myelopathy. Critique The patient has fever. The differential diagnosis includes metastatic cancer. A low serum iron level by itself does not distinguish iron deficiency from anemia of chronic disease. Distortion of the marrow sinuses causes deformity of the erythrocytes and also allows transit of immature myeloid and erythroid marrow cells into the peripheral blood. but the node is small and could be difficult to access. it is very invasive. and splenectomy would not be indicated because preservation of splenic function is desirable. the recent use of corticosteroids as an immunosuppressive agent. and miliary tuberculosis. the history of exposure to tuberculosis. hemolysis. or they are not iron-deficient. infection).population). Similarly. Splenomegaly is consistent with tuberculosis. In this patient the easiest. which yields a diagnosis in 80% of patients with miliary tuberculosis. Although infiltrative myelopathies usually are caused by metastatic cancer. although open-lung biopsy would also yield a diagnosis. Since bronchoscopy with bronchoalveolar lavage yields a diagnosis of tuberculosis in only 30% of patients with miliary disease. the serum ferritin level must also be determined. severe stress (blood loss. safest approach to confirm the diagnosis is bone marrow biopsy with culture. Administration of an oral iron polysaccharide or possibly parenteral iron or addition of ascorbic acid would be correct if the patient were found to be iron-deficient. Therefore. the risk of complicating infection or bleeding would be greater than in bone marrow biopsy. Hematology and Oncology:Question 19 The correct answer is B Educational Objectives Diagnose anemia with low serum iron. Aspiration of an abdominal lymph node likewise could yield a diagnosis of tuberculosis. tuberculosis may be implicated in as many as 5% of cases. Critique There are two main reasons patients do not respond to oral iron therapy: Either they are noncompliant. and a leukoerythroblastic blood smear characterized by immature myeloid cells and the presence of nucleated erythrocytes and teardrop forms. The demonstration of caseating granulomas is strong evidence for tuberculosis or histoplasmosis.

Although the formation of alloantibodies can accelerate hemolysis of transfused blood. To address this risk. Hematology and Oncology:Question 23 The correct answer is A . it is not acceptable first-line therapy and initiating it does not make it safe to continue the heparin therapy. Therefore.diagnosis. donor erythrocytes will have been cleared from the circulation long before the 6-month anniversary of the patients last transfusion. macrocytic anemia. Although antiplatelet therapy may have value in the treatment of heparin-induced thrombocytopenia. Hematology and Oncology:Question 21 The correct answer is A Educational Objectives Recognize the need for prompt diagnosis of red cell aplasia in sickle cell disease. profound nutritional disorders can cause marrow aplasia. they do not adversely affect marrow erythropoiesis. Hematology and Oncology:Question 22 The correct answer is E Educational Objectives Diagnose and treat heparin-associated thrombocytopenia. but the presenting symptoms of severe folate deficiency would be megaloblastic. Moreover. Even when the platelet count has not reached levels below 150. compensatory reticulocytosis is typically maintained. From a practical perspective. Continuation of exposure to heparin can cause lifethreatening cardiovascular thrombotic events. particularly in this patient in need of ongoing anticoagulation for cardiac ischemia. it is essential to discontinue all heparin exposure immediately. Critique The best approach to management of this patient would involve discontinuation of heparin therapy followed immediately by the institution of alternative anticoagulation therapy such as lepirudin or argatroban. Rarely. continuing the heparin infusion regardless of close monitoring or in anticipation of diagnosing the disease on the basis of laboratory studies is not an acceptable alternative. an acute decrease in the platelet count in the setting of ongoing heparin exposure should signal the need to discontinue heparin therapy. There are no other obvious causes for thrombocytopenia. Although splenic sequestration crisis can cause a rapid fall in hemoglobin levels. recent studies suggest that patients with heparin-induced thrombocytopenia have a hypercoagulable state associated with a significant risk of thrombosis that may persist for days to weeks after heparin is discontinued. Critique Infection with parvovirus B19 is a common cause of transient reticulocytopenia in humans. Patients with sickle cell anemia or thalassemia compensate for their chronic hemolytic state through elevated erythropoietic activity and can rapidly develop lifethreatening anemias in the face of parvovirus-mediated erythrocyte hypoproliferation. and a clinical diagnosis of heparin-induced thrombocytopenia should be made.000/μL (or the lower limit of normal for the specific laboratory). discontinuing heparin as the only intervention is not sufficient. This patient with presumptive exposure to heparin during prior peripheral vascular surgery develops acute thrombocytopenia 2 days after being exposed to heparin. After the diagnosis is made.

whereas iron deficiency anemia is also typically associated with elevated transferrin concentration and low serum ferritin concentration. Even though autoantibodies can be detected in the antibody screen. but there is no reason to look for them. Erythropoietin can be used to treat anemia in patients with HIV disease but should generally be considered only after other causes of anemia have been ruled out and . they are used to identify homologous units that have a high likelihood of surviving as long as the autologous cells in circulation. the purpose of this testing is to detect alloantibodies. because iron therapy can promote certain infections and toxicities in HIV disease. Autoantibodies may be present. transfused group O cells are expected to be detectable in circulation after only 3 days. The finding of iron deficiency should prompt a search for blood loss. If an alloantibody had been missed during compatibility testing. in view of the universal compatibility of group O blood. hence the ‘3-day rule’ for compatibility samples. and endoscopy of the gastrointestinal tract should be considered in this regard. Anemia with a low serum iron concentration is characteristic of the anemia of chronic disease and of iron deficiency anemia. iron therapy should not be given to a patient with HIV disease in the absence of iron deficiency. because of the appropriate rise in hemoglobin. The patient’s history marks her as an antibody responder. Critique The blood bank wants a new sample because this patient’s recent transfusion may have stimulated new alloantibody specificities to emerge that would not be present in older samples. There is no reason to suspect hemolysis. studies have shown that anamnestic reactions can produce clinically detectable antibodies in that short a time. staining a bone marrow aspirate for iron is the gold standard for diagnosing iron deficiency anemia. increased red cell distribution width. the serum ferritin concentration may be normal because ferritin is an acute. Although 4 days is not sufficient time for primary alloimmunization to proceed to detectable levels of antibody.Educational Objectives Recognize the need for compatibility blood samples in patients requiring multiple transufsions. and high normal to elevated serum transferrin concentration. When iron deficiency occurs in the setting of inflammation. It is common to use group O cells when antigen-negative units are required. which is the preferred test to identify an ongoing hemolytic reaction. Treatment with iron without identifying the source of blood loss could delay the diagnosis of an otherwise curable gastrointestinal malignancy. Antibody screening does not include a direct antiglobulin test. The anemia of chronic disease is also typically associated with low serum transferrin concentration and normal to elevated serum ferritin concentration. Rather. and there is a higher likelihood that she will make further antibodies upon continued challenge.. In the absence of a hemolytic reaction. but compatibility-testing methods were not designed to screen for them. Critique In this context. Findings consistent with but not diagnostic of iron deficiency in this patient are low mean corpuscular volume. Hematology and Oncology:Question 24 The correct answer is D Educational Objectives Differentiate between iron deficiency anemia and anemia of chronic disease. Furthermore.and chronic-phase reactant. it would be important to look for it only if hemolysis were suspected.

By contrast with the indolent lymphomas. findings on physical examination and CT scan. Although warfarin therapy is likely to be highly effective in reducing the risk of venous thromboembolism. and the risk is compounded in patients with an underlying hereditary thrombotic defect. she should use anticoagulant prophylaxis at times of increased risk. Hematology and Oncology:Question 25 The correct answer is B Educational Objectives Diagnose and treat relapsed lymphoma. and the elevated serum lactate dehydrogenase level are all consistent with relapsed lymphoma in this patient.the serum erythropoietin level is found to be less than 500 IU/mL. HRT has been shown to increase the risk of venous thromboembolism. In these chemotherapy-sensitive patients. Critique Not only should this patient be advised against the use of hormone replacement therapy (HRT). Critique Relapse should be documented by a lymph node biopsy. Aspirin provides minimal if any protection against the development of venous thrombosis. and staging should be completed with bone marrow aspiration and biopsy. Hematology and Oncology:Question 27 The correct answer is A Educational Objectives Recognize and treat drug-induced thrombocytopenia. Critique Determining the cause of hematologic abnormalities may be difficult in transplant recipients. Hematology and Oncology:Question 26 The correct answer is D Educational Objectives Recognize the increased risk of venous thromboembolism associated with hormone replacement therapy. autologous stem cell transplantation has been shown to be superior to salvage chemotherapy alone. particularly in an asymptomatic carrier with an inherited thrombotic defect. Repeating a course of CHOP is not recommended because of the increased risk of doxorubicin-induced cardiotoxicity and the availability of more active salvage regimens. Most patients with relapsed lymphoma respond to second-line salvage chemotherapy. Systemic symptoms. Data from the Women’s Health Initiative indicate that HRT is also not beneficial in reducing the risk of arterial thrombotic complications. and prednisone. Relapse occurs in approximately 50% to 60% of patients with advanced-stage (II-IV) aggressive non-Hodgkin’s lymphoma treated with cyclophosphamide. and treatment should not be delayed. relapsed aggressive lymphomas have a progressive clinical course. because of her history. 40% to 50% of patients who underwent transplantation had long-term disease-free survival. a trial of investigational agents is not appropriate at this time. hydroxydaunomycin. Trimethoprim-sulfamethoxazole is a leading cause of drug-induced . vincristine. Because there are numerous effective second-line chemotherapy regimens for this patient. it is associated with a risk of major bleeding and for that reason is contraindicated.

The use of oral contraceptives confers a fourfold increase in the risk of venous thromboembolism. or consumption disorders such as thrombotic . The hereditary thrombophilias. Discontinuation of trimethoprim-sulfamethoxazole should lead to improvement in the platelet count within 7 to 14 days in most cases—if improvement does not occur. and the most likely cause of thrombocytopenia in this patient. Platelet transfusion would be indicated if the platelet count were lower than 10. especially factor V Leiden mutation. If the peripheral blood smear revealed a microangiopathic process. congenital thrombocytopenias such as the May-Hegglin anomaly.thrombocytopenia. other diagnoses should be considered. is to monitor her platelet count closely and intervene therapeutically if the platelet count drops further. Critique The correct approach to management of this patient. The postpartum period is associated with a higher risk of thrombosis. but may also develop insidiously. as these agents are associated with the development of post-transplant microangiopathy. Because the hemoglobin level and leukocyte count are normal. Hematology and Oncology:Question 29 The correct answer is E Educational Objectives Recognize when to initiate therapy for idiopathic (immune) thrombocytopenic purpura. The differential diagnosis includes hypoproliferative disorders such as myelodysplasia.000/μL or if there were active bleeding. then changing the immunosuppressive regimen or decreasing the doses of cyclosporine and/or tacrolimus should be considered. but it remains a matter of controversy whether other adverse obstetric outcomes such as preeclampsia and intrauterine growth retardation are associated findings. and anticoagulation is generally recommended for a period of 6 weeks. Critique The best management would be to observe her carefully throughout her pregnancy and administer prophylactic low-molecular-weight heparin for 6 weeks after she gives birth. who has asymptomatic thrombocytopenia. Hematology and Oncology:Question 28 The correct answer is A Educational Objectives Recognize the increased risk posed by a prior episode of thrombosis and its implications for pregnancy. soon after beginning the drug. have been associated with a threefold increase of stillbirth in the second and third trimester. Epsilonaminocaproic acid is an antifibrinolytic drug that is sometimes of use in patients with thrombocytopenic bleeding but is not indicated in this patient. reported that the antepartum risk of venous thrombosis was extremely low in pregnant women with a prior thrombotic event in association with transient risk factors. and the peripheral blood smear shows only decreased platelets. but the risk of recurrence is quite low in women with prior thrombosis in association with just such a transient risk factor. The study from Brill-Edwards et al. the likelihood that thrombocytopenia is the result of a primary or secondary marrow process is low and bone marrow examination is not required. Termination of pregnancy would therefore not be appropriate. Therefore observation rather than antepartum administration of low-molecular-weight heparin is appropriate for this patient. Immunosuppressive therapy should not be discontinued because doing so would risk the viability of the allograft. as demonstrated by the presence of schistocytes. The thrombocytopenia usually occurs acutely.

but it is not associated with . is incidental (gestational) thrombocytopenia. Although idiopathic thrombocytopenic purpura cannot be excluded with absolute certainty.microangiopathies. Induction chemotherapy with cytarabine and idarubicin does not play a role in this disease unless there is progression to leukemia. A bone marrow aspiration with biopsy is not required for the diagnosis of idiopathic thrombocytopenic purpura and is unlikely to be of value in an otherwise healthy young woman with no hematologic abnormalities. Headache. Bleeding and thromboembolic events (arterial and venous) represent potentially lifethreatening complications of this disease. Clinical symptoms are the result of hyperviscosity. Hematology and Oncology:Question 30 The correct answer is B Educational Objectives Diagnose and treat polycythemia vera. hypervolemia. Moreover. Patients usually have facial plethora and splenomegaly. dyspnea. pruritus. Most experts would not recommend instituting therapy for idiopathic thrombocytopenic purpura in patients with a platelet count above 30. or idiopathic thrombocytopenic purpura. mild elevation in leukocyte and platelet counts is also common. asymptomatic thrombocytopenia in pregnancy. and night sweats are common. Imatinib mesylate is specific therapy for chronic myeloid leukemia and is not indicated in polycythemia vera. disseminated intravascular coagulation. most congenital thrombocytopenias are associated with platelet morphologic abnormalities that were not observed in this case. phlebotomy is the treatment of choice. Critique This patient has polycythemia vera. Splenectomy is reserved for the late stages of the disease. particularly when it develops in the third trimester. The most likely disorder in a patient of this age who is otherwise healthy and in whom the other hematologic parameters are normal is idiopathic (immune) thrombocytopenic purpura. the most common cause of mild. blurred vision. Therefore. Hematology and Oncology:Question 31 The correct answer is C Educational Objectives Recognize and manage gestational thrombocytopenia. there is no reason to study the patient’s relatives. but the thrombotic risk remains elevated. The pathogenesis of this disorder is not well understood. Platelet aggregation studies are likely to be abnormal simply because of her thrombocytopenia. Genetic causes of thrombocytopenia are usually diagnosed in childhood and would be uncommon as a first diagnosis in a 27-year-old with no suggestive family history.000/μL in the absence of bleeding. and the presence of splenomegaly or an abnormal marrow karyotype other than the Philadelphia chromosome establish the diagnosis of polycythemia vera. Regular phlebotomy to maintain the hematocrit at less than 45% promptly controls the symptoms of the disease. Critique The major differential diagnosis in this patient is between idiopathic (immune) thrombocytopenic purpura and gestational thrombocytopenia. Alcohol use is not associated with polycythemia and abstaining would not affect the course of the disease. and hypermetabolism. they are used to investigate potential causes of platelet dysfunction rather than abnormalities in platelet number. therefore. Elevated red cell mass (or hematocrit values above 60% for men and 56% for women) in the absence of secondary causes of erythrocytosis. for palliation of symptomatic splenomegaly with hypersplenism. Polycythemia vera is characterized by an increased red cell mass.

which are not transmitted through cell-free plasma. Critique The patient has glucose-6-phosphate dehydrogenase (G6PD) deficiency and acute. other diagnoses should be considered. None of these effects is as critical to her health as the prevention of HLA alloimmunization. the actual incidence of these reactions is less than 5% of transfusions. Prevention of primary alloimmunization to HLA antigens can be achieved by leukocyte reduction.adverse maternal or fetal outcomes and therefore does not require specific intervention. G6PD protects cells from membrane oxidation. Leukocyte reduction has no effect on erythrocyte alloimmunization or hemolytic reactions. The peripheral blood smear does not reveal schistocytes. This patient’s history argues against existing HLA sensitization through pregnancy or transfusion. Hematology and Oncology:Question 33 The correct answer is D Educational Objectives Understand the features of glucose-6-phosphate dehydrogenase deficiency. The patient is cytomegalovirus-positive herself. drug-induced hemolytic anemia. In deficient persons. She is at risk for reactivation of her own virus during immune suppression after transplant. therefore there is no evidence of an underlying microangiopathic hemolytic anemia.000/μL or other pregnancy-related complications occur. This patient presents with mild thrombocytopenia in the third trimester of an otherwise uncomplicated pregnancy. If the platelet count drops below 70. therefore transmission of the virus is not a concern. Prestorage leukocyte reduction eliminates donor-derived cytokine elaboration that causes febrile non hemolytic reactions. nor is there an elevated blood pressure that would suggest preeclampsia. Hematology and Oncology:Question 32 The correct answer is B Educational Objectives Recall the purpose of leukocyte reduction. The patient is taking no medications that are associated with drug-induced thrombocytopenia. These cells can harbor pathogens such as cytomegalovirus or HTLV-1. Many adverse reactions to transfusions are related to the presence of donor leukocytes in blood products. The clinical history and physical examination are unremarkable. which would predispose the patient to the development of disseminated intravascular coagulation. There is no personal or family history of prior hematologic disease. In this patient the occurrence of acute intravascular and extravascular hemolysis following administration of primaquine and the presence of Heinz bodies and characteristic bite . Prevention of HLA alloimmunization is critical to her chance of obtaining an organ and stopping dialysis. Critique Donor leukocytes can act as immunizing antigens and are the chief cause of human leukocyte antigen (HLA) alloimmunization. Success in finding a compatible cadaveric organ that will not be rejected acutely through humoral mechanisms is directly related to the presence of HLA class I antibodies. therefore there is also no evidence of an underlying disorder such as sepsis. Current technology for leukoreduction does not remove enough T cells to prevent graft-versus-host disease. membrane oxidation causes cells to become rigid and susceptible to mechanical hemolysis and trapping by the spleen and liver.

The utility of functional ADAMTS 13 assays in diagnosing and monitoring the therapy of this disorder has not been established. CT of the head is often normal. and brain ischemia. although diffuse. Hemoglobin is absorbed into the proximal renal tubules where it is degraded ultimately into hemosiderin. Primaquine is one of a number of drugs that produce acute hemolysis in G6PDdeficient subjects. and microangiopathic hemolytic anemia and an elevated serum lactate dehydrogenase level without another apparent cause should be sufficient evidence of thrombotic thrombocytopenic purpura to institute plasma exchange therapy. Thrombotic thrombocytopenic purpura primarily affects the microvasculature. or protein S are very rarely encountered in this setting. The diagnosis of thrombotic thrombocytopenic purpura is a clinical one. Hematology and Oncology:Question 34 The correct answer is A Educational Objectives Avoid needless radiologic evaluation for an occult neoplasm in an older patient with a first venous thrombotic event. Although functional deficiency of the vWF-cleaving protease. diagnosis and therapy of this disorder should be made on clinical criteria. Continued assay improvement and correlation with clinical disease in humans is required before assays of ADAMTS 13 are accepted for use in clinical . ADAMTS 13. protein C. Critique This patient presents with signs and symptoms consistent with thrombotic thrombocytopenic purpura. which spills slowly into the urine. The presence of a leukoerythroblastic blood smear is consistent with the stress of acute hemolysis and not necessarily a marrow infiltrative process. The detection of low G6PD enzyme activity during an acute hemolytic crisis is often futile because the erythrocytes most susceptible to destruction are those with the lowest enzyme activity (the younger cells have greater enzyme activity and therefore survive). Liver function studies may also be normal in these patients and are not helpful in reaching a diagnosis. Because hemoglobin will be present only in the immediate period of the hemolysis. the detection of urine hemosiderin may indicate a more remote prior episode. Although the factor V Leiden and prothrombin G2021 0A mutations are frequently present in older patients with a first deep venous thrombosis in the absence of a family history. does not generally lead to the development of lesions of sufficient size to be detected by CT. An unprovoked episode of deep venous thrombosis in older patients has been associated with an increased incidence of an occult malignancy. An extensive search for an occult neoplasm in otherwise healthy patients does not always detect a treatable malignancy or is not cost-effective. is common in patients with thrombotic thrombocytopenic purpura. The absence of hemoglobinuria does not necessarily exclude an acute intravascular hemolytic episode. Sensitive screening tests are available for G6PD deficiency but may be abnormal in other hexose monophosphate shunt deficiencies. and these tests are not recommended. Critique No further evaluation is necessary. deficiencies of antithrombin III. therefore. Hematology and Oncology:Question 35 The correct answer is C Educational Objectives Recognize the limitations of laboratory studies in the diagnosis of thrombotic microangiopathy.cells on peripheral blood smear make this diagnosis very likely.

platelet counts generally normalize within 3 to 4 days after surgery. Leukemoid reaction attributable to infection is unlikely in this patient because he does not have fever and lacks other manifestations of infection. and acute myeloid leukemia. and thrombocytosis. The platelet count might . Extracorporeal circulation during coronary artery bypass grafting induces transient thrombocytopenia. CT of the abdomen is not warranted in this case. A balanced translocation between chromosomes 9 and 22 (Philadelphia chromosome) creates a unique gene. hypophosphatasia. Although thrombotic thrombocytopenic purpura may on occasion occur soon after bypass surgery. Critique This most likely cause of this patient’s thrombocytopenia is heparin-induced thrombocytopenia. Hematology and Oncology:Question 37 The correct answer is D Educational Objectives Diagnose heparin-induced thrombocytopenia. Acute bacterial endocarditis can present with neutrophilia with a left shift but is commonly associated with fever and constitutional symptoms that are lacking in this case. which codes a 210-kDa protein (p210) that functions as a tyrosine kinase. Critique Typically.decision making. This kinase was shown to cause a CML-like disease in mice when expressed in hematopoietic cells. weight loss. Although significant neutrophilia can be associated with occult tumors. Idiopathic thrombocytopenic purpura cannot be entirely excluded but does not usually develop so rapidly and would be less common than heparin-induced thrombocytopenia in an elderly patient in this setting. Hematology and Oncology:Question 36 The correct answer is B Educational Objectives Diagnose chronic myeloid leukemia in a patient presenting with neutrophilia. It is likely that the patient developed heparin-induced thrombocytopenia at or immediately after the time of her surgical procedure. It may also be low in other hematologic disorders. the fact that the peripheral blood smear does not reveal increased schistocytes excludes this diagnosis. but a low score is not diagnostic of CML. surgery alone cannot account for this persistent and severe thrombocytopenia. such as paroxysmal nocturnal hemoglobinuria. the lack of constitutional symptoms and weight loss and absence of anemia and occult blood in the stool make an occult gastrointestinal malignancy very unlikely. The leukocyte alkaline phosphatase score is often low in chronic myeloid leukemia. bcr-abl. Therefore blood cultures and echocardiography are not necessary. The Philadelphia chromosome can be detected in peripheral blood mononuclear cells by interphase fluorescence in situ hybridization (FISH) analysis and is diagnostic of CML. splenomegaly. This patients abdominal symptoms can be explained by his splenomegaly. Therefore. given the patient’s normal prothrombin time and partial thromboplastin time. Disseminated intravascular coagulation is relatively unlikely. Furthermore. but it is uncommon for the platelet count to drop by more than 25% because of bypass alone. and no obvious primary disorder that would stimulate its development. Patients may have fatigue. myelofibrosis. chronic myeloid leukemia (CML) is diagnosed on a routine blood count that demonstrates leukocytosis with circulating myeloid precursors.

in some patients. . heparin is no longer present when the disease develops. elevated hemoglobin A2 can be reduced into the normal range). By contrast. The role of heparin-induced thrombocytopenia in her persistent thrombocytopenia is supported by the precipitous decline in the platelet count and development of lower-extremity ischemia after reexposure to heparin. Hematology and Oncology:Question 38 The correct answer is B Educational Objectives Distinguish among different causes of hypochromic microcytic anemia. Glucose-6-phosphate dehydrogenase deficiency would not account for the patient’s signs and symptoms. Although there are not many studies of the therapy of this disorder. An elevation in the hemoglobin A2 level is consistent with β-thalassemia and is one of the chief methods for distinguishing this disorder from mild ct-thalassemia. the mean onset of thrombocytopenia and thrombosis was 9. patients have responded to alternative anticoagulation in a manner similar to those with more standard presentations of heparin-induced thrombocytopenia. it would be associated with a hypoproliferative state (low reticulocyte count) and is not a recognized cause of elevated hemoglobin A2 levels (in fact. Iron deficiency anemia would be unusual in an otherwise well-appearing young man. as well as thrombocytopenia and thrombosis common to standard presentations of heparininduced thrombocytopenia. Iron deficiency could be ruled out by normal plasma iron studies or the presence of stainable iron on bone marrow biopsy. The pathogenesis of this disorder is not well understood. are characteristically both microcytic and hypochromic (low mean corpuscular volume and mean corpuscular hemoglobin values). it did not occur until 19 days afterward.actually have been improving at the time of her read mission. This syndrome is associated with the development of anti-heparin-platelet factor 4 antibodies. although the anemias associated with uncomplicated glucose-6-phosphate dehydrogenase deficiency and sickle cell disease are typically normocytic and normochromic. the anemias of iron deficiency. and the spleen is often not palpable. Furthermore. α-thalassemia. in patients with β-thalassemia who are also iron deficient. It is assumed that the pathologic antibodies recognize conformationally altered platelet factor 4 bound to the platelet surface or to endogenous glycosaminoglycans expressed on the surface of endothelial cells.or β-thalassemia appear well. At such a time she would be expected to be particularly susceptible to recurrent exposure to heparin. Hematology and Oncology:Question 39 The correct answer is C Educational Objectives Diagnose delayed-onset heparin-induced thrombocytopenia. Critique Anemia can result from any of the listed conditions. Critique This patient developed severe thrombocytopenia and an arterial thrombotic event 2 weeks after a hospitalization in which she was given heparin. The presence of this disorder could be confirmed by measuring heparin-platelet factor 4 antibodies using the 14C-serotonin release assay. and β-thalassemia. Patients with mild α. In one study.2 days after withdrawal of all heparin. They have mild elevations in reticulocyte count consistent with their chronic hemolytic disorder. She has delayed-onset heparin-induced thrombocytopenia. as her antibody levels to heparin would be maximal.

The risk of hepatitis C transmission is also extremely low. Retrospective studies. Platelet aggregation studies would be unrevealing. Screening for hepatitis B core antibody can identify some but not all of these donors. on the basis of clinical findings of transient cerebral ischemic episodes in association with the presence of a lupus anticoagulant and a high level of cardiolipin IgG antibodies. Measurements of platelet-associated immunoglobulin G are nonspecific and would not help identify the patient’s disorder. despite a relatively high incidence (0. One of the reasons these two viruses have such a low risk is the use of nucleic acid testing. Critique Hepatitis B poses the highest risk. and the peripheral blood smear is notable for polychromatophilia and occasional spherocytes and rare nucleated erythrocytes. Her history is typical for the disease. indicate that warfarin to a target INR greater than 3 is required to prevent recurrent thrombotic events. and highly sensitive testing. Antiplatelet therapy alone does not adequately protect patients with antiphospholipid syndrome against arterial thrombosis. confidential unit exclusion. Hematology and Oncology:Question 41 The correct answer is C Educational Objectives Administer antithrombotic therapy to a patient with antiphospholipid antibody syndrome and transient cerebral ischemia. there are still donors who are antigen-negative but capable of transmitting the virus.5%) in the general first-time donor population.Genetic assays for factor V Leiden or prothrombin G2021 0A mutation would not account for the patients thrombocytopenia. With the combination of donor health screening. Moreover. the risk of HIV transmission from asymptomatic donors is negligible. Although testing for hepatitis B surface antigen was initiated in the late 1970s and has been constantly refined and improved. Hematology and Oncology:Question 42 The correct answer is A Educational Objectives Diagnose and treat warm antibody autoimmune hemolytic anemia. Critique This patient has warm antibody autoimmune hemolytic anemia. Recent data however indicate that an INR of 2 to 3 is adequate for preventing recurrent venous thrombosis. as they would not test platelet function in the presence of heparin. . which comprise for the most part patients with antiphospholipid antibody syndrome and underlying collagen-vascular disease. Critique Lupus anticoagulants are acquired defects that are associated with arterial as well as venous thrombotic events. Laboratory studies reveal a decreased hemoglobin and elevated reticulocyte count. these genetic defects are associated with venous rather than arterial thrombosis. technical difficulties have delayed nucleic acid testing for hepatitis B. Hematology and Oncology:Question 40 The correct answer is D Educational Objectives Recall the risks of viral transmission through transfusion. Unfortunately. which relies on polymerase chain reaction technology to amplify viral material. and her physical findings of jaundice and mild splenomegaly are also consistent with this diagnosis. The diagnosis of antiphospholipid antibody syndrome can be made in this patient.

these findings are consistent with increased erythrocyte destruction in the periphery. Patients older than 60 years or patients with a history of thrombotic events are considered to be at high risk for thromboembolic complications. Busulfan therapy is associated with significant risk of secondary leukemias and does not have a role in management of essential thrombocythemia. Warfarin anticoagulation should be avoided in this patient because her essential thrombocythemia puts her at high risk for bleeding complications. Treatment with a nonmutagenic agent such as anagrelide. Critique This patients liver disease gives evidence of poor synthetic function. The risks of donor exposures preclude prophylactic transfusion of fresh frozen . and use of hydroxyurea to reduce the platelet count below 400. and the use of exogenous erythropoietin is of uncertain benefit.000/μL significantly reduces this risk. it can be anticipated that excessive hemorrhage due to impaired hemostasis will be a complication during major surgery. Essential thrombocythemia may be difficult to distinguish from other myeloproliferative syndromes. The cause of his recurrent venous thrombosis is unclear. Hematology and Oncology:Question 43 The correct answer is C Educational Objectives Diagnose and treat high-risk essential thrombocythemia. However. Therefore aspirin therapy alone would not be sufficient for this patient. 1 mg/kg daily. Diagnosis is made in patients with platelet counts in excess of 600. Hematology and Oncology:Question 44 The correct answer is A Educational Objectives Recognize the indications for transfusion of fresh frozen plasma.22) excludes chronic myeloid leukemia.Taken together. The appropriate initial therapy for this patient is prednisone. which blocks megakaryocyte maturation. Although the results of the coagulation tests are only slightly abnormal. and absence of marked marrow fibrosis excludes myelofibrosis. Erythropoietin may be of value in certain hypoproliferative anemias or in patients with anemias secondary to exogenous influences such as chemotherapy. Intravenous immunoglobulin and danazol may have activity in some patients with autoimmune hemolytic anemia but are not firstline agents. splenectomy should be reserved for patients who are corticosteroid-resistant. but there are no randomized data regarding reduction of thrombotic events. Imatinib mesylate is specific therapy for chronic myeloid leukemia and is not indicated in essential thrombocythemia. with a compensatory bone marrow response. A hemoglobin level of 13 g/dL or lower excludes polycythemia vera. Critique Essential thrombocythemia is the most common myeloproliferative disorder affecting middle-aged and older adults and is characterized by thrombotic and hemorrhagic complications. Likewise. would also reduce the platelet count. to which 65% to 80% of patients respond.000IjiL in the absence of known causes for reactive thrombocytosis. absence of t(9. Low-dose aspirin therapy is beneficial in the treatment of ischemic neurologic and vision symptoms and erythromelalgia in essential thrombocythemia. endogenous erythropoietin levels are elevated in patients with autoimmune hemolytic anemia.

Prednisone. Heparin. Arthroscopic surgery is. Critique The incidence of symptomatic venous thrombosis after arthroscopic surgery is not nearly as high as that after total knee replacement. Splenectomy should not be routinely done if the patient responds initially to prednisone. and the presence of petechiae suggests microvascular bleeding. Hematology and Oncology:Question 46 The correct answer is C Educational Objectives Administer appropriate therapy for a symptomatic distal deep venous thrombosis after arthroscopic surgery. would be the most appropriate initial management. Approximately 10% to 15% of patients may have resolution of their disease or achieve stable platelet counts (greater than 30. Intravenous immunoglobulin and intravenous anti-Rh(D) are both highly effective therapies for idiopathic thrombocytopenic purpura. a risk factor. This patient’s low-molecular-weight heparin should be withheld the day before surgery to allow for the elimination of the anticoagulant effect. Also because of the risks of transfusion. but because of cost are generally reserved for patients whose thrombocytopenia is severe or who do not achieve a stable platelet count in response to a low dose of prednisone (less than 5 to 10 mg/d). The anticoagulant effect of warfarin can be temporarily corrected by fresh frozen plasma by supplying factors that are synthesized with normal gamma carboxylation. In general. behaves as an inhibitor and its effect is not corrected through fresh frozen plasma administration. no other diagnostic studies are required to initiate therapy. and venographic studies have shown an incidence of 4% to 17% after such procedures. Although the use of splenectomy in the treatment of idiopathic thrombocytopenic purpura remains in evolution. however.000/ μL) on a low dose of prednisone. however. simple observation would not be appropriate. Critique The presumptive diagnosis in this patient is idiopathic thrombocytopenic purpura. the hemostatic effect of fresh frozen plasma lasts only as long as the biologic life of the shortest factors being replaced. 1 mg/kg daily. For example. nor would fresh frozen plasma be effective until the drug effect was diminished. either splenectomy or the use of other agents such as intravenous immunoglobulin or intravenous anti-Rh(D) could be considered if the patient either does not respond to prednisone or requires unacceptably high doses to maintain a safe platelet count. hypovolemia and hypoproteinemia are best treated with sterilized plasma derivatives such as albumin or with plasma expanders. therefore. factor VII has a steady-state half-life of approximately 6 hours. Symptomatic calf vein thrombosis that has been objectively documented should be treated with therapeutic doses of low-molecular- .plasma merely to treat elevated coagulation findings. Hematology and Oncology:Question 45 The correct answer is E Educational Objectives Begin appropriate initial therapy for idiopathic thrombocytopenic purpura. however. fresh frozen plasma is not. Albumin may transiently correct hypoproteinemia caused by malnutrition or decreased synthesis but is usually not effective if the protein deficiency is due to nephrosis or capillary leak. required because of the low-molecular-weight heparin. Therapy is indicated because the platelet count is very low.

would be a prophylactic rather than a therapeutic regimen for venous thromboembolism in this setting.weight heparin. 30 mg subcutaneously every 12 hours. “bridging” therapy . There is no indication for placement of an inferior vena cava filter. Platelet transfusion as initial therapy in thrombotic thrombocytopenic purpura has been reported to worsen the microangiopathy. Therefore. if it is feasible to treat the patient entirely in the outpatient setting. Some hematologists believe that corticosteroids used early in thrombotic thrombocytopenic purpura can hasten resolution. caused by autoantibodies or enzyme defects. as long as he is reasonably active. because the beneficial effect is related to what is being given. Hematology and Oncology:Question 47 The correct answer is B Educational Objectives Select the proper plasmapheresis treatment for thrombotic thrombocytopenic purpura Critique This patient has thrombotic thrombocytopenic purpura and should be treated by plasmapheresis with fresh frozen plasma. Enoxaparin. therefore it is not recommended to use anti-inflammatory agents and to obtain a repeat ultrasound in 1 week. but only as an adjunct to fresh frozen plasma infusion and plasmapheresis. Daily plasma exchange with fresh frozen plasma allows an entire blood volume to be replaced with normal plasma while avoiding hypervolemia and is the standard of care for this disorder. followed by warfarin for a period of 3 months. Plasmapheresis with the usual saline and albumin replacement is not used for thrombotic thrombocytopenic purpura. Because this patient had adequate therapy after a first deep venous thrombosis. Hematology and Oncology:Question 48 The correct answer is B Educational Objectives Prevent venous thromboembolism after major orthopedic surgery in a patient at high risk for recurrent thrombosis. such as inability to undergo therapeutic anticoagulation or clot extension at a therapeutic level of anticoagulation. relapses are common if plasma exchange is discontinued prematurely. Whether platelets can be safely used to treat bleeding once a patient’s thrombotic thrombocytopenic purpura has started to respond to plasma exchange remains controversial. Thrombocytic thrombocytopenic purpura is one of the few disorders that requires fresh frozen plasma as pheresis replacement. Critique Anticoagulation prophylaxis should be continued for 6 weeks. his risk of recurrence would be quite low during the several weeks between cessation of warfarin and the orthopedic surgery. This syndrome has been linked to disturbances in the macromolecular structure of factor VIII. often with fatal outcome. in addition to what is being removed. Therapy should be continued until well after the hemolysis has subsided and the lactate dehydrogenase level and platelet count have returned to normal. Approximately 20% of patients develop clot extension if anticoagulation is not undertaken.

with scleroderma. Negative biopsy results do not rule out underlying graft-versus-host disease. vitiligo. scarring eczematoid rash. Laxative abuse is unlikely and does not explain skin changes. Pseudomembranous colitis is uncommon with trimethoprim-sulfamethoxazole therapy. however. lichen planus. Administration of lowmolecular-weight heparin after knee replacement surgery for as long as 4 weeks has not been conclusively shown to reduce the rate of deep venous thrombosis. Critique Approximately 50% to 70% of the recipients of allogeneic stem cell transplantation develop some manifestations of chronic graft-versus-host disease. If this patient had not had cardiac disease. Recurrence of chronic myeloid leukemia would not cause diarrhea.with low-molecular-weight heparin or intravenous unfractionated heparin would be unnecessary in the days immediately before surgery. diarrhea. Critique Except in the case of cardiovascular compromise. and may contribute to some ventilation-perfusion mismatches. Increases in red cell mass will not improve lung function. is associated with bloody diarrhea. myocardial contractility. as compared with 7 to 10 days of therapy. Transfusion of red cells can improve hypotension and hypovolemia. Biopsy of the rectal or colonic mucosa may be helpful in confirming the diagnosis and eliminating other potential causes such as cytomegalovirus colitis. Many physicians now allow patient hemoglobins to drift to low levels. Therefore it is recommended that anticoagulant prophylaxis be continued for 6 weeks after surgery rather than just 1 week. the red cells would not have been indicated. Most patients are on ventilators because of airway or alveolar dysfunction and not because of poor oxygen transport. Red blood cell transfusion is indicated in order to raise the hematocrit of this patient with cardiac disease. Anorexia. Hematology and Oncology:Question 49 The correct answer is A Educational Objectives Recall the indications for red cell transfusion. weight loss. and other responses. often found in stool culture. Hematology and Oncology:Question 50 The correct answer is C Educational Objectives Recognize the clinical manifestations of chronic graft-versus-host disease. and abdominal pain are predominant gastrointestinal manifestations of the disease. hyperpigmentation. greater than average because of his previous episode. and symptomatic patients should be treated with corticosteroids empirically. Shigella. Hematology and Oncology:Question 51 The correct answer is C . malabsorption. Aspirin is not a recommended agent for prophylaxis of deep venous thrombosis after major joint replacement. and hair loss. Hypoxia from a pulmonary cause is not usually improved by transfusion. The patients risk of postoperative venous thromboembolism is. most patients can compensate for reductions in red cell mass through increased stroke volume. but red cells are not as efficient at increasing oncotic pressure as colloids or plasma expanders. with an absolute threshold around 7 g/dL as the point where myocardial oxygen consumption is impaired strictly by low red cell mass. The risks of donor exposure are not warranted simply to increase circulating volume. Skin involvement is common.

despite her protein S deficiency.Educational Objectives Treat venous thrombosis associated with transient risk factors. Myelofibrosis is commonly associated with splenomegaly. Neither low fixed doses of warfarin nor the combination of aspirin and clopidogrel has been shown to be efficacious for the prevention of recurrent venous thromboembolism. Febrile. By contrast. Hematology and Oncology:Question 52 The correct answer is C Educational Objectives Recognize and differentiate myelodysplastic syndrome from other causes of anemia. Passive acquisition of donor leukoagglutinins causes transfusion-related acute lung injury. these include pregnancy and a caesarean section. leukocyte reduction prevents or alleviates these reactions. Although a history of diverticulosis and aspirin use suggests possible bleeding and iron deficiency. Critique Although this patient has protein S deficiency and a strong positive family history of venous thromboembolism. The symptoms are due to the pyrogens associated with the bacteria. They are characterized by peripheral blood cytopenias and dysplasia of erythroid. The mean corpuscular volume is commonly elevated. not fever and hypotension. and the time sequence make anaphylaxis unlikely as a cause of his symptoms. Critique Myelodysplastic syndromes are clonal hematopoietic stem cell disorders that occur predominantly in patients older than 50 years. it is appropriate to discontinue oral anticoagulation therapy after 3 to 6 months at a target INR of 2 to 3 and employ it prophylactically in high-risk situations only. It is most common in platelet concentrates that are 4 to 5 days old—long enough for log-phase growth to occur in the inoculum. Group AB individuals have no ABC isoagglutinins and are therefore “universal” plasma/platelet donors. Angiotensin-converting enzyme (ACE) inhibitors are rarely associated with blood dyscrasia. Vitamin Bi 2 deficiency can cause all the laboratory abnormalities described except reduced segmentation of neutrophils. Hematology and Oncology:Question 53 The correct answer is A Educational Objectives Recognize the presentation of a bacterial contamination reaction. nonhemolytic reactions from antibodies to donor leukocyte antigens are common but rarely cause hypotension and vascular collapse. and the findings described do not occur with drug-induced cytopenias. the low leukocyte and platelet counts. her first thrombotic event occurred in association with several transient risk factors. and the recipient’s inflammatory response. toxins elaborated during their growth. Critique Bacterial contamination of platelets can occur when skin plugs enter the blood during needle insertion or because of asymptomatic donor bacteremia. Therefore. and findings on peripheral blood smear do not support a diagnosis of iron deficiency anemia. there is hypersegmentation in vitamin Bi 2 deficiency. This patients fever. the fact that he has no respiratory distress or wheezing. granulocytic. but respiratory distress and hypoxemia are the hallmarks of this reaction. high mean corpuscular volume. Hematology and Oncology:Question 54 The correct answer is D . or megakaryocytic lineages.

Therapy for 6 weeks will result in a higher recurrence rate than therapy that lasts 3 to 6 months. Clearly. or 24 hours before warfarin initiation.0. Hematology and Oncology:Question 55 The correct answer is B Educational Objectives Determine the appropriate duration of anticoagulant therapy for a patient with a postoperative deep venous thrombosis. How to alter a patients anticoagulation therapy from argatroban to warfarin has been a confusing issue to clinicians.Educational Objectives Determine a schedule by which to alter a patient’s anticoagulation therapy. although it is monitored by using the partial thromboplastin time. Argatroban. Therefore the recurrence risk will be quite low. The rate increases to 25% to 30% at 1 month if extended prophylaxis is not employed. Approximately 30% of pregnancies are associated with low levels of HLA alloimmunization.0. Critique The appropriate period of anticoagulation for a symptomatic post-operative deep venous thrombosis is 3 months. the incidence remains at 10% to 20% as assessed by bilateral venography after 7 to 10 days of therapy. Discontinuing argatroban at the time warfarin is initiated. A loading dose of warfarin is unnecessary. the vitamin K-dependent coagulation inhibitor). a schedule that called for argatroban to be discontinued when the INR reached 2. affects the INR even when used alone. initiation of therapy at high doses is associated with an increased incidence of bleeding complications. to ensure that a full anticoagulant effect of warfarin is achieved before argatroban is discontinued. it does not hasten the achievement of a stable therapeutic INR any more than does therapy initiated at a dose of 5 mg/d. Critique It would be best to initiate warfarin at 5 mgld. It is also recommended that the argatroban infusion be reduced to 2 μg/kg perminute before warfarin is initiated. and anticoagulation for 1 year or for the rest of her life is not warranted.0 would not allow enough time for the desired anticoagulation effect of warfarin to be achieved. the amount of time required for the anticoagulant effect of warfarin to be achieved. Both argatroban and warfarin prolong the INR. would leave the patient without anticoagulation therapy for 3 to 4 days. Hematology and Oncology:Question 56 The correct answer is E Educational Objectives Diagnose platelet refractoriness. If it is not in the therapeutic range of 2 to 3. Some experts also recommend that argatroban be continued for at least 3 days after starting warfarin. presumably through stimulation from placental tissue as . argatroban should be restarted and continued until the INR attrributable to warfarin alone is in the therapeutic range. this patient’s thrombosis was provoked by the joint replacement surgery. and discontinue argatroban when the INR is greater than 4. Therefore. Furthermore. Critique The refractoriness observed in this patient is most probably caused by alloimmunization. The INR should then be rechecked 4 to 6 hours after argatroban is stopped. Furthermore. it would expose the patient to the transient procoagulant effect of warfarin that can occur soon after therapy is initiated (owing to a rapid fall in the levels of protein C. Although low-molecular-weight heparin is effective in reducing the rate of deep venous thrombosis after total hip replacement. it is recommended that argatroban be discontinued only after the INR is greater than 4.

Mutations in other genes (TfR2. they are a major cause of platelet refractoriness. Although moderate thrombocytopenia can develop through loss of platelets during vigorous bleeding and through the dilutional effect of massive transfusion. often within minutes of a transfusion and many times without evidence of a transfusion reaction. the low platelet count is evident before delivery. and hereditary or acquired sideroblastic anemias. usually obtained from a single donor through apheresis. Given the patients ancestry and clinical picture. Nonimmune causes do not usually lower the platelet increment until well after 1 hour post-transfusion. but the womans immune system retains the ability to form an anamnestic response when rechallenged. Disseminated intravascular coagulation can lower platelet counts. Although these alloantibodies do not themselves cause thrombocytopenia (because they do not destroy autologous platelets). which can have multiple causes. β-thalassemia/hemoglobin E. Homozygosity for the C282Y mutation in the HFE gene occurs in approximately 1 in 200 persons of northern European ancestry and is the most common cause of iron overload in whites.well as unapparent fetal-maternal hemorrhage. The post-transfusion platelet increment was less than 25% of the expected rise for someone of average body size and blood volume. This patients catastrophic hemorrhage is a nonimmunologic complication of pregnancy. Nontransfusional iron overload also occurs in certain anemias characterized by high degrees of ineffective erythropoiesis. Just like patients who have received many transfusions. The elevated serum ferritin concentration and high transferrin saturation levels are consistent with iron overload. In the HELLP (hemolysis. FPN 1) that lead to hereditary iron overload have recently been reported. he could have iron overload related to 3-thalassemia intermedia or sideroblastic anemia. The best discriminator of immune and nonimmune platelet destruction is the measurement of the immediate post-transfusion platelet count. multiparous women are at risk of having a high level of circulating HLA antibody. hemoglobin H disease. Mild thrombocytopenia of nonimmune origin can occur in many pregnancies. unmatched platelet transfusions are usually ineffective. and the patient requires specially ordered matched platelets. but almost always causes abnormalities in the tests of clotting function. Dietary iron overload is common in blacks from rural southern Africa and may have a genetic component. The high serum indirect bilirubin and low serum haptoglobin levels in association with the normal reticulocyte count are evidence of ineffective erythropoiesis. The observed poor platelet count increment in this patient suggests platelet transfusion refractoriness. Hematology and Oncology:Question 57 The correct answer is B Educational Objectives Diagnose and treat iron overload Critique The most effective procedure to assess the possibility of iron overload in this patient is a diagnostic liver biopsy. these mechanisms usually do not cause transfusion refractoriness. These HLA antibodies often decay to subdetectable levels quickly. elevated liver enzymes. When immune refractoriness exists. Immune destruction occurs quickly. A quantitative phlebotomy program is an alternative way to document iron overload but would be difficult to perform in an anemic patient and does not inform about the . and low platelet count) syndrome. Such anemias include β-thalassemia major and intermedia. but the patients normal platelet count prior to delivery rules out this complication.

and toxicity of imatinib mesylate are under investigation. Because there is no evidence of progression to acute leukemia. because approximately 10% of patients with HELLP syndrome ultimately meet criteria for . elevated liver function tests. blood counts become normal. Treatment with desferrioxamine should be instituted only in an anemic patient with confirmed iron overload. Some authorities consider it a variant of preeclampsia. particularly if the cost of imatinib mesylate is prohibitive. and if not discontinued because of toxicity can result in 5-year survival rates of 50% to 70%. Hematology and Oncology:Question 59 The correct answer is B Educational Objectives Recognize and initiate therapy for the HELLP syndrome Critique This patient has the HELLP (hemolysis. Transient leukopenia. this patient can be successfully treated with imatinib mesylate (Gleevec). and infections. Rash. Hydroxyurea is commonly used to normalize blood counts quickly and results in a 5-year survival of 35% to 50%. Despite her advanced age and multiple medical problems. Allogeneic bone marrow transplantation would not be an appropriate treatment option. because the toxicity of the agent is markedly increased if iron stores are not increased. metabolic complications. examination findings. Demonstration of a balanced translocation between chromosomes 9 and 22 (Philadelphia chromosome) confirms the diagnosis.presence of iron-related hepatic damage. In 90% of patients treated with a daily oral dose of imatinib mesylate. and muscle cramps are common side effects of therapy with imatinib mesylate. and 30% to 40% of the patients no longer have detectable CML cells in their bone marrow. especially given the patient’s age and history of cardiac problems. Hydroxyurea is well tolerated and could be an alternative therapy for this patient. The optimal duration of therapy. Daily subcutaneous interferon-alfa injections are more effective in decreasing CML cells in the bone marrow. mild nausea. there is no need for systemic induction chemotherapy. Without therapy. interferon-alfa therapy would be poorly tolerated and would not be a good choice for this patient. Hematology and Oncology:Question 58 The correct answer is E Educational Objectives Treat chronic myeloid leukemia in an elderly patient. and laboratory test results are consistent with chronic-phase chronic myeloid leukemia (CML). Staining a bone marrow aspirate for iron is the gold standard for diagnosing iron deficiency but is not reliable for diagnosing iron overload. and fluid retention may also occur. long-term benefits. low platelets) syndrome. Critique This patients presentation. this patient is at risk of developing hyperleukocytosis and circulatory compromise. hepatotoxicity. However. progressive splenomegaly.

As with preeclampsia. Other myeloproliferative syndromes have to be excluded in order to ascertain the diagnosis. ABC-incompatible acute intravascular hemolytic reactions can cause symptoms like those of this patient. Furthermore. teardrop cells. however. and dry bone marrow aspiration. Hematology and Oncology:Question 61 The correct answer is B Educational Objectives Diagnose an allergic transfusion reaction. however. There is no role for aggressive diuresis or antiplatelet agents in the management of established preeclampsia or HELLP syndrome. Bacterial contamination can lead to respiratory distress and shock. even before overt hemolysis is evident. because fresh frozen plasma is stored frozen and used within hours of thawing. Disseminated tuberculosis is also associated with marrow fibrosis. The units given were group AB. a diagnosis of HELLP syndrome does not require hypertension and proteinuria. Although this patient has circulating myeloid precursors. hepatomegaly. Critique This patient is having an acute anaphylactic transfusion reaction. but fever is often present. As this case illustrates. are most consistent with the HELLP syndrome. Critique Myelofibrosis is characterized by splenomegaly. Hematology and Oncology:Question 60 The correct answer is E Educational Objectives Recognize myelofibrosis. and leukoerythroblastosis is uncommon. which must be considered in the differential diagnosis of this patient’s disorder. Plasma exchange is indicated for thrombotic thrombocytopenic purpura. absence of an elevated leukocyte count and FISH analysis that is negative for t(9. Hairy cell leukemia can present with pancytopenia. Her presentation and laboratory studies. circulating erythroblasts and myeloid precursors. definitive therapy for HELLP syndrome should be focused toward urgent delivery of the fetus. therefore the recipient’s naturally . after which the disorder usually resolves. therefore ABC plasma incompatibility cannot be the cause. and antiplatelet agents may also increase the incidence of thrombocytopenic bleeding complications.22) exclude chronic myeloid leukemia. However. The splenomegaly and hepatomegaly result from extramedullary hematopoiesis. and hypotension make it more than a severe systemic allergic reaction. caseating or noncaseating granulomas are typically observed on bone marrow biopsy. There is no role for intravenous immunoglobulin in management of HELLP syndrome or preeclampsia. bronchospasm.preeclampsia. and marked bone marrow fibrosis. Patients with myelodysplastic syndromes can have varying degrees of marrow fibrosis. but massive splenomegaly and hepatomegaly are uncommon. Randomized trials of prophylactic aspirin to decrease the incidence of preeclampsia have not shown a benefit to treatment. normocytic anemia. Peripheral blood smear in this disorder shows characteristic hairy mononuclear cells. The presence of mucosal edema. splenomegaly. there is insufficient time for any contaminating bacteria to grow to a sufficient inoculum size. There are typically severe dysplastic abnormalities in one or more lineages in blood and marrow of patients with myelodysplastic syndrome. Fresh frozen plasma contains virtually no intact red cells.

however. Heterozygosity for the prothrombin gene mutation is a definite risk factor for an initial episode of deep venous thrombosis in whites. reduce the number of long-term responders to approximately 65%. are most often transient.5 to 2 will provide significant protection against recurrent venous thromboembolism without a significant risk of major bleeding. have not shown the recurrence risk to be any greater in patients with an identifiable thrombophilic defect than in those without one. Hematology and Oncology:Question 63 The correct answer is D Educational Objectives Identify patients with idiopathic thrombocytopenic purpura in whom anti-Rh(D) therapy is not appropriate. it is believed to act largely by blocking uptake of antibody-coated platelets by Fc receptors in the spleen. The incidence of recurrence in patients with a first unprovoked episode of deep venous thrombosis after adequate treatment (3 to 6 months of anticoagulation at therapeutic doses) is 5% to 15% per year. and she should encourage her first-degree relatives to be tested for the mutation. an anti-CD20 monoclonal antibody. most studies. Initial therapy of idiopathic thrombocytopenic purpura with corticosteroids leads to a remission rate of 70% to 80%. Intravenous immunoglobulin is an acceptable agent to use in refractory idiopathic thrombocytopenic purpura after splenectomy and probably the most likely of the agents mentioned to induce a response. It is not efficacious in splenectomized patients. and allergic reactions like this patients urticaria and rash are uncommon. Rituximab. although ultimately only 15% to 25% of these remissions can be sustained on an acceptable dosage of corticosteroids. The use of intravenous anti-Rh(D) would not be indicated in this patient. Patients taking angiotensin-converting enzyme inhibitors may have sudden acute hypotension due to disordered bradykinin metabolism. is associated with a response rate of 25% to 50% in patients with refractory idiopathic . most of which occur in the first year after splenectomy. Critique This patient has idiopathic thrombocytopenic purpura that has proved refractory to corticosteroid therapy and splenectomy. although relapses. anti-Rh(D). Responses to intravenous immunoglobulin. Although the mechanisms of this agent in idiopathic thrombocytopenic purpura are complex. Second-line therapy of idiopathic thrombocytopenic purpura may include intravenous immunoglobulin. although recent data from the PREVENT trial indicate that anticoagulation at a target INR of 1. Hematology and Oncology:Question 62 The correct answer is B Educational Objectives Recognize the risk posed by the prothrombin G20210A mutation.occurring anti-B has no cellular target in the group AB plasma. Lifelong anticoagulation is generally not undertaken for an uncomplicated first episode of deep venous thrombosis. Splenectomy induces remission in approximately 80% of such patients. Critique The prothrombin G2021 0A gene mutation is a definite risk factor for an initial episode of deep venous thrombosis. however. The hallmark of transfusion-related acute lung injury is hypoxemia from pulmonary leukoagglutination and alveolitis rather than bronchospasm and edema. or a number of other approaches. but these reactions are quick to resolve and cause few other symptoms. splenectomy.

and the radiation field should be carefully examined regularly each year after treatment. for example. The false-negative rate of positron emission tomography scans in this setting is not yet known with certainty. It is not known to cause fibrosarcoma. occurring at another time) cancers can develop from a variety of causes. The positron emission tomography scan was unhelpful because it did not indicate abnormal uptake in the liver nodules. Some organs are susceptible to multiple metachronous tumors. but their effect may require several weeks to become apparent. Therapeutic radiation is associated with a 1% per year risk of a second solid tumor within the radiated field for at least 25 years after treatment. Critique It is first necessary to determine whether the nodules in the liver are benign or malignant by percutaneous needle biopsy. Tamoxifen is associated with an increased risk of endometrial cancer and a decreased risk of a second breast cancer and colon cancer. Patients who receive therapeutic radiation. are at increased risk. Measuring serum carcinoembryonic antigen (CEA) would not be helpful in any case. Radiation therapy alone would be inappropriate if liver metastases were confirmed because palliative chemotherapy would be the preferred approach. colon and prostate cancer in men or colon and breast cancer in women. similar risk factors do not account for these metachronous cancers. People cured of cancer remain at risk for other cancers. Danazol and cyclophosphamide are also of benefit in some patients. Second (and third) metachronous (that is. even in very small total doses. Hematology and Oncology:Question 64 The correct answer is B Educational Objectives Recognize the late complications of breast cancer treatment. for example. Typically the majority of these cases involve two common forms of cancer. therefore. it would be obviated if the nodules in the liver were found to be malignant. because it would deny or delay potentially curative surgery. therefore. such as bladder cancer and breast cancer. head and neck cancer patients are at increased risk of lung cancer and both kinds of tumor occur with increased frequency in tobacco and alcohol abusers. Fibrosarcoma is a rare tumor. It is not clear whether the risk diminishes with longer time periods after therapy. Hematology and Oncology:Question 65 The correct answer is C Educational Objectives Confirm staging test results before precluding surgery for non-small-cell lung cancer. it is unlikely to be completely unrelated to the breast cancer and its treatment. Surgery with curative intent should be offered if the non-small-cell lung cancer appears localized to the area near the left hilum. Approximately 15% of patients who are cured of a first cancer by a surgical procedure (no chemotherapy or radiation therapy) develop a second neoplasm in another organ. Chemotherapy would be inappropriate if the liver nodules are benign (outside of a clinical trial). Some tumors occur in distinct organs but have very similar risk factors. Fibrosarcoma is not a tumor that routinely occurs as multiple metachronous tumors. CEA may be elevated in localized or metastatic lung cancer. Fibrosarcoma does not share risk factors with breast cancer. especially because . Critique This patients previous radiation therapy is the likely cause of her fibrosarcoma.thrombocytopenic purpura.

Daily vitamin supplementation may improve other health outcomes. operative mortality is significantly lower at a center where many such procedures are performed than at centers with limited experience.faint activity can be interpreted as positive or negative. it is associated with acute proctitis in 18. Incontinence is more common with surgically treated patients (severe urinary incontinence in 9. Hematology and Oncology:Question 67 The correct answer is D Educational Objectives Recognize the effects of radiation therapy in patients treated for clinically localized prostate cancer. There is no evidence that supplementation with β-carotene has any effect on the risk of head and neck cancer. CT results indicate that the patients tumor may be resectable. and impotence is common in both groups. Thrombosis occasionally occurs in the postoperative period. but is not known to cause lung or head and neck cancers. Hematology and OnCology:Question 69 The correct answer is A Educational Objectives Recognize which screening tests are associated with the strongest evidence of reducing the mortality rate in cancer Critique . The best option is referral of the patient to a center with surgical expertise in the management of pancreatic cancer.6 % vs. but there is no evidence that this strategy will reduce cancer risk. depending on the radiologist.7% of patients. Critique The patient should avoid excessive use of alcohol. Futhermore. Critique This case is a classic presentation of pancreatic cancer. Establishing a definitive histologic diagnosis is not necessary before the referral. and it is not commonly related to radiation therapy. Hematology and Oncology:Question 68 The correct answer is D Educational Objectives Make an appropriate referral for a patient with potentially resectable pancreatic cancer. Benzene exposure is known to be a causative factor for leukemias. Because the patient does not have symptomatic jaundice. emergency biliary drainage is not indicated. Tobacco and alcohol abuse appear to be synergistic as causative agents for head and neck cancer. in a patient with potentially resectable disease. because surgical resection offers the only potential for long-term survival. a factor that has been linked to squamous cell carcinoma of the head and neck. 3. Critique Radiation therapy to the prostate gland has significant morbidity. for example. Hematology and OnCology:Question 66 The correct answer is A Educational Objectives Recognize modifiable risk factors related to cancer of the head and neck.5 %).

it was estimated that approximately five to ten deaths from colorectal cancer would be averted for every serious complication or postoperative death from the screening-treatment process (Robinson et al). however. there was no decrease in the rate of death from lung cancer. A federally sponsored randomized trial of sigmoidoscopy versus a control arm in more than 150. no major organizations recommend routine screening for lung cancer. It is uncertain whether FOBT decreases the incidence as well as the mortality from colorectal cancer. even though more operable lung cancers were detected and survival time from diagnosis of lung cancer was increased in the screened group. which showed an even greater reduction in the number of deaths from colorectal cancer (Mandel et al). but results are unlikely to be known for several years. The recommendation is supported by well-performed case-control studies that show an association between sigmoidoscopy within the previous 10 years and lower risk of death from colorectal cancer. Pap smears do detect a large number of noninvasive lesions such as lowgrade squamous cell intraepithelial lesions and atypical squamous cells of unknown significance. Because Pap smears target preneoplastic lesions of the cervix. A randomized study is ongoing to determine if some of these low-grade lesions can be monitored without definitive therapy. nonsmoking woman with adenocarcinoma of the axilla. Because this study design relies on prior choice to undergo the procedure and people who choose to undergo screening tests may have better underlying health than those who do not. Most North American professional organizations recommend the use of sigmoidoscopy in screening for colorectal cancer every 5 years. These observations are most likely due to overdiagnosis—detection of non-life-threatening cancers—by the screening tests. and if HPV testing can aid in the decision. The reduction appears to be similar whether or not FOBT slides are rehydrated. the test is in large measure a form of primary prevention. Therefore. its findings represent an intermediate strength of evidence. the effects of human papillomavirus (HPV) DNA testing and Pap smears have never been tested in a randomized trial. Critique In a young. virtually every other study design has shown a substantial decrease in the incidence of cervical cancer as well as mortality associated with institution of Pap smears. even of current or former cigarette smokers. breast cancer . although the false-positive rate of the tests is increased by rehydration.All three randomized trials of biennial fecal occult blood testing (FOBT) reported to date have shown a decrease in colorectal cancer mortality with screening compared with controls. In one of the three trials of biennial screening. To date. results have been inconsistent. One of the trials had an annual screening arm. case-control study designs do not allow for a direct comparison of benefits and harms such as perforation or bleeding.000 volunteer participants is therefore being conducted. Furthermore. whose natural history is ill defined. In a randomized trial of chest radiography plus sputum cytology every 4 months in male cigarette smokers. Hematology and OnCology:Question 70 The correct answer is A Educational Objectives Evaluate adenocarcinoma presenting in an isolated axillary lymph node. aimed at lowering the incidence of invasive cancer. In the case of Pap smears.

fever. frequently increasing suspicion or providing evidence of benign conditions. The tissue from biopsy should be stained for HER2/neu. otherwise. It is also used in the screening of women with BRCA-1 or BRCA-2 germ-line abnormalities. such as evaluation of a patient who has no evidence of breast cancer but has axillary lymph nodes containing adenocarcinoma. duration of antibiotic therapy. a negative mammogram is insufficient reason to discontinue thorough evaluation of a breast abnormality. The presence of a central venous port removes this patient from the low-risk category. Critique One of the most common causes of malpractice in the United States is failure to diagnose breast cancer at an early stage. although helpful in selected patients with metastatic disease. MRI of the breast is probably more sensitive than mammography. . Several studies have demonstrated that prognosis and the degree of therapy required are both directly related to the size and stage at diagnosis. his disease might be treated with oral antibiotics. Options include modified radical mastectomy. and some patients and clinicians might prefer modified radical mastectomy. total cost for managing the febrile neutropenic episode. which are in turn directly related to early or delayed detection. Proceeding with resection alone would not be advised in such a young patient to whom potentially curative options are available. adjuvant chemotherapy would be recommended. therefore. adding vancomycin or removing the central venous port is not indicated. it is reasonable to continue the same therapy. The sensitivity of mammography is 75% to 90%. Although often used in clinical practice. or infectionrelated mortality. Hematology and Oncology:Question 71 The correct answer is A Educational Objectives Evaluate a breast mass. positive results would confirm the diagnosis of breast cancer. Ultrasonography is the procedure of choice for this patient. Hematology and Oncology:Question 72 The correct answer is A Educational Objectives Treat febrile neutropenia in a patient with cancer. Optimal therapy for breast cancer should be initiated. In either case. but the specificity of MRI is quite low and this technique is reserved for special circumstances. No identifiable risk factor (other than being female and over 40 years of age) can be found in 75% of all patients with newly diagnosed breast cancer. which identifies an occult primary tumor 30% to 50% of the time. Because this patient’s condition is stable. Although use of radiation therapy is certainly one option. Critique A thorough re-assessment should be performed to determine the causative agent. It can be very helpful in evaluating a palpable abnormality of the breast. Measurement of circulating tumor markers. For this reason. or radiation therapy for breast conservation. and the neutrophil count is increasing.remains the most likely diagnosis. colony-stimulating factors are not recommended because results of randomized trials have failed to demonstrate consistent improvements in the duration of hospitalization. is insufficiently sensitive or specific to be of use in evaluating a suspicious breast mass. especially because breast cancer is the most treatable type of adenocarcinoma (even with curative intent in this case). it is not the only option.

unexplained weight loss of more than 10%) or dyspnea on exertion from a mediastinal recurrence that limits lung function. some centers were administering mantlefield radiation therapy with anteriorly weighted ports such that two thirds of the radiation was administered to the front and one third to the back. particularly sites of bulky disease. drenching night sweats in the previous month. Hematology and Oncology:Question 74 The correct answer is C Educational Objectives Recognize androgen ablation as the cornerstone of treatment in metastatic disease . Pulmonary fibrosis can occur in patients treated with bleomycin plus radiation therapy. Thus. The delivery of therapeutic doses of radiation therapy to the anterior mediastinum includes large segments of the coronary arteries. recurrent disease is most likely to be detected by the development of B symptoms according to the Ann Arbor criteria (unexplained fever. Hodgkins disease usually recurs in previously involved sites. This method produced a high rate of constrictive pericarditis that has since been dramatically reduced by administering the radiation therapy in equal doses front and back. The onset of constrictive pericarditis from radiation therapy in this setting was usually insidious and associated with progressive exercise intolerance. and nearly all the rest occur 1 to 5 years after treatment. Hematology and Oncology:Question 73 The correct answer is B Educational Objectives Recognize the long-term complications of mediastinal radiation therapy. Constrictive pericarditis is also unlikely given the time period (15 years ago) of this mans treatment. and dacarbazine plus radiation therapy that any case would be reportable. not by myocardial ischemia. Critique The most likely diagnosis is exercise-related myocardial ischemia in this young man. Therefore. blemoycin. The Stanford group documented a threefold increase in the rate of fatal myocardial infarction in their patients receiving mantlefield radiation therapy. fatigue.Intravenous ceftazidime remains the best choice. and exercise intolerance. are dyspnea on fibrosis and shortness of breath. it is important that patients who receive mantle-field radiation therapy as a component of their treatment for Hodgkin’s disease be informed of the increased risk of myocardial infarction and counseled about the importance of maintaining a healthy weight and lowering cholesterol with a statin as prophylaxis. presenting symptoms would include pallor. Twenty to 30 years ago. Furthermore. and the radiation exposure promotes premature atherosclerosis. however. The presenting signs of pulmonary fibrosis. Furthermore. Recurrent Hodgkins disease is exceedingly unlikely because the disease rarely recurs this late after diagnosis. Half of all cases of relapse of Hodgkin’s disease occur within the first year of completing treatment. vinblastine. Secondary marrow failure is so rare after doxorubicin.

Hematology and Oncology:Question 76 The correct answer is C Educational Objectives Recognize the relative risks and benefits of hormone replacement therapy (HRT) and tamoxifen to women at increased risk for breast cancer.) . most authorities agree that symptomatic patients should be treated with androgen ablation. Outside of a clinical trial. Chemotherapy with docetaxel or mitoxantrone. cardiac disease has also been similar in the treatment and control groups. The risk of ischemic heart disease in the BCPT was similar in the tamoxifen and placebo arms.2% over the next 5 years for this patient. was an absolute risk of at least 1 . adjuvant combined-modality therapy should be offered to patients with stage II and III (regional lymph node involvement) disease who otherwise have good performance status. Critique This patient should stop taking the HRT and start taking tamoxifen. Critique This patient has stage II rectal cancer. It would be reasonable to encourage her to participate in a clinical trial for breast cancer prevention. Therefore. Because the tumor has penetrated the bowel wall. such therapy should be used only after disease progression when the patient is receiving androgen blockade. however. the efficacy of tamoxifen for the prevention of breast cancer is not known for women who are concurrently being treated with HRT. Although the timing of the commencement of therapy is somewhat a matter of controversy. and radiation therapy may be used in the palliation of advanced disease.66% over the next 5-year period. Adjuvant therapy involving systemic chemotherapy and concurrent pelvic radiation therapy have been shown to reduce the risk of local recurrence and increase survival. she is at risk of developing a local recurrence. although the total number of ischemic heart disease events was small. women had to have stopped taking HRT at least 3 months before randomization (and remain off it for the duration of the trial). The BCPT showed that administration of tamoxifen for as long as 5 years decreased the relative risk of breast cancer by approximately 50%. (In therapeutic studies of tamoxifen for established breast cancer.of the prostate. Although regular colonoscopy is a reasonable surveillance strategy. Critique Since the 1940s. such as the STAR (Study of Tamoxifen and Raloxifene) trial. In order to enter the BCPT study. Postoperative radiation therapy alone is inferior to the combination of chemotherapy and radiation therapy. representing an absolute risk of approximately 5. Her risk of developing breast cancer is substantially elevated. Hematology and Oncology:Question 75 The correct answer is B Educational Objectives Treat rectal cancer with adjuvant chemoradiation therapy. corticosteroids. even though no lymph nodes are involved. androgen ablation has been the cornerstone of treatment in metastatic prostate cancer. Chemotherapy may palliate this patient’s bone pain and fatigue. a placebo-controlled randomized trial of tamoxifen. The breast cancer risk criterion that qualified women for the Breast Cancer Prevention Trial (BCPT).

Critique The patient presents with signs of intestinal obstruction. In that trial. it would not be prudent to watch and wait under these circumstances. Because the rate of progression cannot be accurately predicted. The effect of estrogen alone on heart disease is still under study in the WHI trial. for example. The results of a carcinoembryonic antigen test would not be available for a few days. however. the MRI should be obtained promptly. in which increased uptake is dependent on either an osteoblastic reaction or new bone deposition. and MRI is currently the diagnostic test of choice. rectal stricture is unlikely. Hematology and Oncology:Question 77 The correct answer is D Educational Objectives Recognize the potential emergency of spinal cord compression in a cancer patient with new-onset back pain. Left untreated. The patient does not have signs of an acute abdomen. Critique Spinal cord compression must be ruled out in a patient with cancer who presents with back pain of new onset. but it is not the procedure of choice in this situtation. because it causes endometrial cancer. appear to increase the risk of ischemic heart disease in postmenopausal women. spinal cord compression associated with malignancy can progress to myelopathy over a period of weeks. The study was therefore halted because the harms of progestin plus estrogen were believed to outweigh the benefits in the preventive setting. there is no immediate need to administer dexamethasone before establishing the diagnosis. therefore a surgical consultation can be deferred until additional diagnostic studies have been done to identify the cause of the obstruction. Because the patient does not have any neurologic symptoms. Possibilities include either malignant obstruction (peritoneal carcinomatosis. CT scanning might be helpful in determining the degree of bone destruction and whether bone or tumor is impinging on the cord. and . Because colonoscopy done 1 year earlier was unremarkable. another colonoscopy is unlikely to be helpful. Nondiagnostic plain radiographs do not rule out the diagnosis. but estrogen alone is not generally given to women with an intact uterus. Hematology and Oncology:Question 78 The correct answer is E Educational Objectives Diagnose intestinal obstruction in a patient treated for rectal cancer. and the current radiographic studies suggest that the obstruction is at the level of the small bowel. In the Women’s Health Initiative (WHI) trial. CT scanning is the most useful diagnostic study to distinguish among the possibilities.Use of estrogen plus progestin HRT does. HRT also increased the relative risk of breast cancer by 26%. Most patients with spinal cord compression have pain that is localized to the spine or radicular in nature. and the most urgent need is to define the cause. Therefore. It is more sensitive and specific than a gastrointestinal series and shows more of the intraabdominal anatomy. the relative risk of coronary heart disease was elevated by 29% in the HRT arm. which studied the use of estrogen plus progestin by healthy postmenopausal women. or metachronous primary tumor) or nonmalignant obstruction (caused by radiation enteritis or adhesions). Because the distal colon and rectum are not filled with stool. nor do bone scans.

No trials have demonstrated that doing so provides any benefit to the patient. One of these is whether they should undergo special studies routinely to detect asymptomatic. and the disease follows a more indolent course. of which bronchoalveolar cell carcinoma is considered a subtype.would have little influence on management of this case. The false-positive rate of ultrasononography of the uterus is high in asymptomatic women. Approximately 40% of women taking tamoxifen will develop endometrial thickening as assessed by ultrasound examination. routine restaging with chest radiographs and hepatic imaging failed to result in improved survival or quality . Therefore. Tamoxifen taken to prevent breast cancer increases the risk of endometrial cancer approximately two. Hematology and Oncology:Question 79 The correct answer is E Educational Objectives Appropriately monitor a patient taking tamoxifen. the absolute risk is approximately 2 to 3 per 1000 women per year. Critique Women taking tamoxifen should be carefully questioned about abnormal gynecologic symptoms such as bleeding and discharge and given a careful pelvic examination. despite the increased risk of endometrial cancer in women receiving tamoxifen. Most of the endometrial cancers diagnosed in these women involve such symptoms as dysfunctional uterine bleeding or discharge. Critique Breast cancer survivors have special concerns. Palliative chemotherapy is usually offered when patients become symptomatic. The tumor cells of bronchoalveolar cell carcinoma are better differentiated than those of the usual adenocarcinoma. reserving diagnostic work-up for those with symptoms or physical abnormalities. Critique This is a classic presentation of bronchoalveolar cell carcinoma. however. leading to a high rate of invasive testing and attendant risk of uterine perforation. Hematology and OnCology:Question 81 The correct answer is B Educational Objectives Monitor a patient after primary and adjuvant systemic therapy for breast cancer. The diagnostic yield of routine endometrial cytologic sampling or biopsy for the detection of early endometrial cancer in asymptomatic women taking tamoxifen is too low to warrant the pain and risk of the procedure.to threefold in postmenopausal women with an intact uterus. Overt metastases to other organs are much less common for this type of cancer at presentation. The fact that the patient is an older woman who is a nonsmoker suggests she has a type of adenocarcinoma. occult metastases. routine screening tests such as ultrasound and biopsy or endometrial aspiration are not indicated. and most are localized (International Federation of Gynecology and Obstetrics stage I) and surgically curable. Infiltrates and nodules are often seen. The disease is usually bilateral when multifocal or metastatic. Hematology and Oncology:Question 80 The correct answer is A Educational Objectives Recognize the clinical presentation of bronchoalveolar cell carcinoma. Many patients live for months without requiring palliative therapy. and two prospective randomized trials demonstrated that frequent.

pylon infection. doxorubicin. Tumor progression after H. Hematology and Oncology:Question 83 The correct answer is D Educational Objectives Diagnose and treat gastric mucosa-associated lymphoid tissue (MALT) lymphoma. the value of such testing is questionable at best. If the tumor does not regress within a year after eradication of the organism and the absence of the organism is confirmed and persistent. switching to a second-line chemotherapy regimen is reasonable. CHOP is generally not considered the first choice of therapy in the setting of gastric MALT lymphoma. In about half the cases of gastric MALT lymphoma associated with H. however. it is now established that estrogen replacement therapy with progesterone modestly increases the risk of a new primary breast cancer. eradication of the infection induces a permanent complete remission. the proliferation of the tumor has become dissociated from the presence of the H. nor in patients with metastases to liver and lungs. Women who are rendered postmenopausal by treatment require special counseling regarding sexual dysfunction and prevention of osteoporosis. pylon antigens. In half of these patients. The fluorouracil. chemotherapy can be used effectively to treat the disease. and the treatment of choice for localized nodal or extranodal diffuse large B-cell lymphomas is four cycles of CHOP plus involved-field radiation therapy. Therefore. Hematology and Oncology:Question 82 The correct answer is C Educational Objectives Recognize the role of second-line systemic chemotherapy in patients with metastatic colorectal cancer. Hematology and Oncology:Question 84 . pylon. hydroxydaunomycin. Although estrogen is the most effective treatment for these symptoms. and she wants to continue receiving therapy. Oxaliplatin has recently been approved for use in combination with infusional 5fluorouracil and leucovorin in patients whose colorectal cancer has progressed while they were taking irinotecan given with 5-fluorouracil and leucovorin. There is no established role for marrow-ablative high-dose chemotherapy regimens in the treatment of patients with metastatic colorectal cancer. preferably in the context of a clinical trial. vincristine. and prednisone (CHOP) is effective therapy for some lymphomas. Cyclophosphamide. Because this patient’s performance status is good. Critique The first course of therapy should be eradication of the Helicobacter pylon organisms. Critique Data from randomized clinical trials have shown that second-line chemotherapy regimens using drugs with established activity in colorectal cancer are superior to best supportive care in patients with good performance status whose cancer has progressed on chemotherapy.of life. mitomycin (FAM) regimen has been used in patients with gastric carcinomas but is not used in lymphoma. pylon eradication could indicate acceleration in its natural history and transformation to diffuse large B-cell lymphoma. because this form of lymphoma appears to be strongly influenced by antigens that are components of H. Prospective studies have not shown metastatectomy to be beneficial for patients with more than three liver lesions. The main role of surgery in this patient would be diagnostic. Total gastrectomy is not indicated in the management of gastric lymphoma. and harmful at worst.

However. If this woman had a larger tumor or many positive lymph nodes. may be slightly more effective. Aromatase inhibitors. Palliative chemotherapy offers modest prolongation of overall survival (8 to 16 weeks). Because smoking cessation is the only proven way to decrease smokers’ risk of dying of lung cancer. Critique The antidepressant bupropion has been shown to double the smoking cessation rate at 1 year in a placebo-controlled trial of cigarette smokers. like those of chemotherapy. but only in patients with adequate performance status. In a patient with such a favorable prognosis. and their physicians do not believe this small benefit outweighs the risks. Although chemotherapy reduces these odds. given her excellent prognosis. . the proportional reduction in recurrence for a woman in her 60s is no more than 15%. these therapies have toxicities and therefore must be applied judiciously. Chest wall radiation has been shown to decrease both local recurrences and systemic recurrences by 65% and 25%.The correct answer is C Educational Objectives Treat breast cancer with adjuvant radiation and systemic therapy. are outweighed by the risks. prescribing bupropion is the best option for this patient. Hematology and Oncology:Question 85 The correct answer is E Educational Objectives Recognize the limitations of palliative chemotherapy in metastatic non-small-cell lung cancer Critique The patient’s performance status would be the limiting factor. Hematology and Oncology:Question 86 The correct answer is C Educational Objectives Recommend interventions of proven benefit in decreasing the risk of lung cancer death in smokers. due in part to widespread application of adjuvant systemic therapy. A recently reported prospective randomized trial has suggested that the combination of tamoxifen and anastrozole is no better than tamoxifen alone. and long-term side effects are not known. and it may be worse than anastrozole alone. The chances that her disease will recur and that she will die over the next 10 years are no more than 10%. Critique Overall mortality from breast cancer has been declining over the past 10 years in the Western world. and most large trials even suggest decreased survival for these patients with poor performance status who are exposed to cytotoxic agents. and the choice of drugs can be modulated by factors such as jaundice. respectively. but they appear to increase the risk of osteoporosis and bone fractures. Patients in bed more than 50% of the day derive no benefit from palliative chemotherapy. patients. Patients whose status is adequate can be offered chemotherapy. these unknown risks are not worth what must be a very small incremental benefit for anastrozole. This patient has a very good prognosis. like anastrozole. Tamoxifen is the drug of choice in this situation. However. chest wall radiation would be appropriate. Hypercalcemia can be treated with bisphosphonates (intravenous pamidronate or zoledronate) concomitant with chemotherapy. the benefits of chest wall radiation. Most guidelines panels. of whatever they were destined to be.

Therefore. Furthermore. A number of studies have been undertaken. Critique This patient with mild hyponatremia is newly diagnosed with small-cell lung cancer. or screening to detect and cure small tumors. Because he has an established diagnosis of small-cell lung cancer. there was no decrease in the number of late-stage lung cancers. . there was a trend toward increased lung cancer recurrence and lung cancer death in subjects who were smokers at study baseline. it did not improve the overall survival rate. In the Mayo Lung Project. Several randomized trials are in progress to test the net benefits and harms. Combination chemotherapy would be the appropriate treatment. and therefore initiating nonspecific therapy such as fluid restriction with or without demeclocycline therapy is not warranted. In a study of patients who had been treated for early-stage non-small-cell lung cancer. there was no decrease in lung cancer mortality in the screened arm. sputum cytology has not been shown to decrease the rate of death from lung cancer in smokers. The most important therapeutic intervention is to treat the underlying cancer. Spiral CT has been shown to detect early-stage lung cancers. 13-carotene supplementation is probably contraindicated for the prevention of lung cancer in smokers. the incidence and mortality rate of lung cancer were increased in the 13carotene study groups. Although isotretinoin was shown to decrease the incidence of second primary tumors in patients treated for a primary squamous cell cancer of the head and neck. Hematology and Oncology:Question 87 The correct answer is D Educational Objectives Recognize the presenting features of hyponatremia of malignancy and the association with small-cell lung cancer.Potential strategies to decrease the risk of lung cancer death in a patient who is having difficulty stopping smoking include more intensified smoking cessation efforts. those randomized to receive isotretinoin did no better than those randomized to receive placebo. He has no symptoms that are clearly attributed to the hyponatremia (the fatigue may also be due to the underlying cancer). confirming that the laboratory studies meet the criteria for syndrome of inappropriate diuretic hormone secretion. In randomized trials. ruling out other causes such as adrenal insufficiency and discontinuing drugs that might possibly lead to free water retention (such as thiazide diuretics) is for academic rather than practical reasons. and the effect on lung cancer mortality is unknown. but the false-positive rate is very high. Furthermore. monitoring of cigarette smokers by sputum cytology plus chest radiography every 4 months for 6 years was compared with usual care as a control arm. on the basis of current evidence. Although more surgically resectable cancers were detected in the screening arm. In placebo-controlled randomized clinical trials of 3-carotene to prevent lung cancer in smokers. medication to limit the cellular or DNA damage of the tobacco carcinogens.

There is no established role for ovarian cancer screening. Over-the-counter. Hematology and Oncology:Question 90 The correct answer is A Educational Objectives Improve the quality of life for breast cancer survivors. including an annual rectovaginal pelvic examination. Because this patients family history gives no evidence of ovarian cancer and because she is young. in particular fluoxetine. Apart from age. but the most successful therapies in estrogen receptor-positive breast cancers are antiestrogenic (selective estrogen receptor modulaters and aromatase inhibitors). Familial cancer syndromes account for only 5% to 10% of all cases. ovarian cancer in a first-degree relative is the most important risk factor for ovarian cancer. even though it provides clear-cut benefits in regard to preventing recurrence of incurable cancer. nonhormonal. although not always. helpful in facilitating intercourse. it is now established that estrogen replacement therapy with progesterone modestly increases the risk of a new primary breast cancer. Critique This patient has testicular carcinoma as manifested by a mass and abnormal markers. The next appropriate step would be to perform an inguinal orchiectomy. Sympathetic counseling is appropriate but not sufficient. Critique Recently reported pilot and prospective randomized clinical trials have demonstrated that selective serotonin reuptake inhibitors. because both his serum ct-fetoprotein and β -human chorionic gonadotropin levels are elevated. To date. no prospective randomized trials have demonstrated that estrogen replacement therapy increases the risk of breast cancer recurrence or death. There is no need to screen for BRCA-1 and BRCA-2 because the family history shows no evidence of this genetic mutation. Retroperitoneal lymph node dissection or combination chemotherapy might be appropriate after a diagnosis is made and clinical staging is complete.Hematology and Oncology:Question 88 The correct answer is D Educational Objectives Initiate treatment of testicular carcinoma. compared with similar reductions in only 20% to 30% of patients taking placebo. she should be reassured. Pelvic irradiation is not indicated in the management of patients with newly diagnosed nonseminomatous germ cell tumors. Although estrogen is the most effective treatment for these symptoms. He cannot have pure seminoma. reduce the incidence and severity of hot flushes by more than half in 50% to 75% of patients. Symptoms may lessen or resolve with time but sometimes they persist throughout treatment with tamoxifen. Postmenopausal symptoms are quite common in women taking tamoxifen after chemotherapy. Critique This patient should be given standard medical care. Hematology and Oncology:Question 89 The correct answer is B Educational Objectives Recognize the most important risk factor for ovarian cancer. venlafaxine. nongreasy vaginal preparations (such as Replens® and Astroglide®) are frequently. and paroxetine. Atrophic vaginitis and dyspareunia as a consequence of menopause can be so severe that women sometimes discontinue taking tamoxifen. .

however. Critique Patients with small-cell lung cancer of limited or extensive stage should be offered prophylactic cranial irradiation. Most patients are asymptomatic. The digital rectal examination is not as sensitive as a PSA screen. therefore a lack of symptoms does not exclude the possibility of an elevated PSA. Hematology and Oncology:Question 91 The correct answer is B Educational Objectives Understand the role of screening for prostate cancer Critique The incidence of prostate cancer increased dramatically when PSA screening began to be used.to threefold. It is hoped that the Prostate.0 ng/mL. At this time. and response to chemotherapy is the key to survival. he should be advised of the significant morbidity that could accompany the treatment of early-stage prostate cancer. Additional chemotherapy. Screening does lead to the earlier detection of prostate cancer. Hematology and Oncology:Question 92 The correct answer is A Educational Objectives Order appropriate further treatment for patients with small-cell lung cancer who achieve clinical complete remission. Lung and Ovary randomized cancer screening trial will answer that question. like unopposed estrogen. Critique Systemic chemotherapy should be offered to this patient because he has symptoms related to his cancer and his performance status is still reasonably good. Tamoxifen has estrogenic agonist properties in the uterus. tumors that will be detected on digital rectal examination in patients with a PSA lower than 4. This patient should be counseled that prostate cancer can be detected earlier with screening. This modest relative risk translates to only a small absolute risk. and. and routine uterine screening is therefore not recommended. Hematology and Oncology:Question 93 The correct answer is D Educational Objectives Recognize when palliative resection of the primary tumor should be considered in a patient who presents with metastatic colon cancer. and patients with breast cancer are reluctant to take them unless nonhormonal therapies are ineffective. Although symptoms such as pelvic discomfort. Surgery is rarely considered for small-cell lung cancer because micrometastatic disease is almost always present. In addition. even with other agents. A meta-analysis of 7 randomized trials evaluating this therapy in patients who achieve a complete response demonstrated 15% to 21% improvement in disease-free and overall survival at 3 years. There are. increases the risk of uterine cancer two. Colorectal.Therefore. or vaginal bleeding should be thoroughly evaluated. it is unclear whether screening will lead to a change in prostate cancer mortality or overall mortality rates. Clinical . most oncologists are unwilling to prescribe estrogens. but it is not known whether earlier treatment would improve his longevity. pain. this patients dyspareunia is a symptom of vaginal estrogen depletion and does not suggest endometrial cancer. has not been shown to benefit complete responders with limited-stage disease.

Regional chemotherapy to the liver is not indicated because the patient’s disease is not confined to the liver. Hematology and Oncology:Question 95 The correct answer is E Educational Objectives Understand the role of adjuvant therapy in completely resected non-small-cell lung cancer. and would delay the initiation of systemic therapy. time to symptom progression. Neither the age of the patient nor the PSA nadir has been shown to affect the length of time to metastatic disease in this population. resection of the primary tumor would not affect his overall survival. Critique The natural history of a rise in PSA levels after radical prostatectomy is poorly understood. in terms of time to disease progression. . would subject him to the risks of surgery. Because systemic chemotherapy is not curative. Therefore.) found that patients with a recurrent rise in PSA level within 2 years of surgery. and overall survival. Randomized trials have failed to show any survival advantage as yet for any adjuvant chemotherapy. with a late. There is no evidence that patients with recurrent disease treated with a prostatectomy have an outcome different from patients treated with other primary therapies. best supportive care can be considered if the patient chooses not to receive chemotherapy. There is no evidence to suggest that combined regional and systemic chemotherapy is beneficial in patients with metastatic disease involving the liver and extrahepatic sites. This patient.trials have shown that immediate institution of systemic 5-fluorouracil-based therapy is superior to best supportive care in the treatment of metastatic colorectal cancer. were at the greatest risk for the development of metastatic disease. with a high Gleason score and a rapid PSA doubling time. The fact that 30% to 50% of patients like this one will develop metastatic disease within a few years makes them good candidates for clinical trials involving novel therapies (such as immunomodulators or vaccines). The patient has no symptoms of obstruction and has only mild anemia. The best series (Pound et al. slow rise in his PSA level. with or without standard chemotherapy. Critique A 50% to 70% cure rate with surgery alone would be predicted for early-stage disease. is at low risk for the rapid development of metastatic disease. Hematology and Oncology:Question 94 The correct answer is C Educational Objectives Recognize the risk factors for metastasis in patients with a biochemical failure after surgery for prostate cancer. Meta-analyses confirm that additional radiation therapy confers no benefit in this setting and in fact may be deleterious to survival in earlystage disease. The indications for palliative resection of the primary tumor in a patient with metastatic disease are obstruction (existing or imminent) and bleeding.

6.91 to 2.Hematology and Oncology:Question 96 The correct answer is A Educational Objectives Treat advanced ovarian cancer.86). Changes in serum lipid levels appear to be a poor surrogate for the impact of hormonal manipulations on ischemic heart disease. but to date has not been shown to offer an advantage over paclitaxel in first-line therapy of advanced ovarian cancer. The BCPT showed no increase in the incidence of any cancer other than uterine endometrial cancer.23 per 1000 in the placebo arm (risk ratio of 3. hormone replacement therapy (HRT) with estrogen plus progestin has recently been shown to increase the risk of coronary artery disease in healthy postmenopausal women despite the fact that HRT lowers serum lipid levels.27). Docetaxel is another taxane. An important life-threatening adverse outcome associated with tamoxifen was an increased risk of deep venous thrombosis or pulmonary embolism. 95% CI 1. however. irrespective of underlying risk of depression (on the basis of medical history obtained at study baseline). given her prior hysterectomy. The BCPT found no difference in the incidence of depression between patients taking tamoxifen or placebo. respectively (RR 1. when used in the cancer prevention setting. The rate of pulmonary embolism was 0. There have been anecdotal reports of depression and mood disorders in association with tamoxifen.15 to 9. presumably not at risk for endometrial cancer. other than reduction of breast cancer risk. . The rates of deep venous thrombosis were 1.84 versus 0. The addition of intraperitoneal chemotherapy may be beneficial in patients with optimally debulked stage III disease (no tumor larger than 1 cm) after completion of systemic chemotherapy.69 per 1000 women in the tamoxifen arm versus 0. For example.34 versus 0. but this approach requires second-look laparotomy to confirm miminal residual disease and is not recommended if the patient has adhesions or the treatment center is not experienced with regional therapy. 95% CI 0.01. More aggressive approaches should be recommended only in the context of a clinical trial.5. This patient is. Critique The Breast Cancer Prevention Trial (BCPT) compared 5 years of tamoxifen use versus placebo in women at elevated risk for breast cancer. Although tamoxifen decreases plasma cholesterol levels. Critique Randomized controlled trials have established that a combination of either cisplatin or carboplatin with paclitaxel is the standard of care for the treatment of advanced ovarian cancer (stage III/IV). Hematology and Oncology:Question 97 The correct answer is B Educational Objectives Know the effects of tamoxifen. it has not been shown to have a statistically significant effect on ischemic heart disease rates in women with or without a history of heart disease.84 per 1000. with a risk ratio of about 2.

Patients with ovarian cancer can have long-term disease-free survival at 10 years. Management involves debulking surgery. recurrence of breast cancer is quite high in women who do not receive radiation therapy. women with lupus dermatitis) are also poor candidates for breast-conserving therapy. breast-conserving therapy and the therapy involved in the latter. given her negative hormone receptors. even if no ovarian mass is evident. Therefore. Ovarian cancer rarely spreads to bone. Patients who have previously had mediastinal or ipsilateral chest radiation (for example. There is no established role for positron emission tomography scan in this setting. the risk of in-breast recurrence for patients who receive radiation therapy is less than 10%. There are. whereas that for lumpectomy alone exceeds 40%. the standard of care mandates debulking surgery first. and a false-positive scan should not preclude a curative approach with surgery and chemotherapy. women with a history of Hodgkin’s disease) and patients at high risk of toxicity from radiation (for example. This patient appears to be an ideal candidate for breast-conserving therapy. In most studies. Several randomized trials have demonstrated that even with systemic therapy. because most recurrences in the breast are treated by mastectomy. . They include the presence of grossly unresected breast cancer. some relative contraindications. mostly related to an unacceptably high risk of subsequent recurrence of breast cancer (>15% over 5 to 10 years). which would be completed with radiation therapy. The presence of widespread ductal carcinoma in situ is also a high risk factor for recurrence in the breast. she is unlikely to benefit. followed by combination chemotherapy. Critique When a woman presents with adenocarcinoma and diffuse peritoneal involvement. however. Because this patient has positive axillary lymph nodes.Hematology and Oncology:Question 98 The correct answer is B Educational Objectives Treat adenocarcinoma presenting with diffuse peritoneal involvement in a woman. Critique Breast-conserving (or preserving’) therapy is as effective as and cosmetically more acceptable than mastectomy. even with this presentation. relatively large lesions. and relatively small breasts. there is no role for tamoxifen. Hematology and Oncology:Question 99 The correct answer is D Educational Objectives Understand which patients with newly diagnosed breast cancer should have mastectomy vs. and a bone scan would not be considered unless symptoms of bone involvement were evident. Radiation therapy plays no role in this setting. Although combination chemotherapy with a taxane and a platinum agent is an important treatment for this woman. an initial course of radiation therapy will result in fewer mastectomies in the long run. therapy should be directed toward ovarian cancer.

his total protein level is increased in the face of a normal albumin level. which would produce microcytic. immunologic. . Iron deficiency anemia is most often noted in the setting of chronic bleeding. because lymphadenopathy in young people generally has a benign cause. Hematology and Oncology:Question 101 The correct answer is A Educational Objectives Recognize the various nonspecific manifestations of multiple myeloma Critique The patient is older than 40 years. hypochromic anemia. however. In addition. the cause is chronic blood loss and the mechanism is iron deficiency. has unexplained anemia. renal dysfunction. Referral to an otolaryngologist might be indicated because smoking-related head and neck cancers can present with lymphadenopathy in the absence of an obvious mucosal lesion. Small-cell lung cancer could produce similar generalized symptoms but is rarely associated with hypercalcemia and is not associated with recurrent urinary tract infection. Anemia is not a common feature of chronic pyelonephritis until renal failure develops. renal failure. Needle aspiration is not the diagnostic procedure of choice in a patient with undiagnosed lymphadenopathy because it fails to obtain adequate tissue for histologic. and recurrent infections: This scenario is common in patients with multiple myeloma. the size of the node (greater than 2 cm in diameter). The age of the patient tends to favor observation as the standard approach.Hematology and Oncology:Question 100 The correct answer is E Educational Objectives Treat lymphadenopathy in a young smoker. and genetic analysis. The diagnosis of myeloma should be considered in any patient older than 40 years with unexplained anemia. and the presence of systemic symptoms. heighten the suspicion of more serious illness. Serum protein electrophoresis would document the presence of a monoclonal gammopathy and he would need to be staged with a bone marrow evaluation (to quantitate marrow plasma cells) and skeletal survey (to search for lytic bone lesions). When cancer of the colon is associated with anemia. The supraclavicular location. Watching and waiting would not be appropriate under these circumstances. bone lesions. There is no proven role for empiric use of antibiotics in the management of a patient with unexplained lymphadenopathy. Critique A chest radiograph is indicated because of the dyspnea on exertion and the night sweats. The patient’s age. or recurrent infections. however. It would also be necessary to measure his serum 32 -microglobulin level. and the fact that she abstains from alcohol make head and neck cancer unlikely. of which this patient has no history or symptoms. meaning that the gamma globulin fraction is increased.

Critique A recently reported prospective randomized clinical trial in patients with metastatic breast cancer has demonstrated that the combination of trastuzumab and paclitaxel are at least additive. she is very unlikely (less than 10%) to respond to endocrine therapy of any sort. Few if any patients are cured of metastatic breast cancer. especially because microscopic metastases are almost always present when an overt metastasis is demonstrated on chest radiograph. systemic therapy probably does result in modest survival prolongation of several months to a few years compared with best supportive care. Given that she has symptomatic visceral disease. however. Patients treated with this combination had significantly longer survival than those treated initially with chemotherapy alone. Therefore. Therefore. even though she will suffer more side effects from it. First. surgery is considered if there is reasonable curative intent. however. The steroid hormone receptors in her tumor. In the four options presented. Critique Surgical therapy is offered in two situations for patients with non-small-cell lung cancer and obvious metastasis. Second. this patient should receive treatment. in women with HER2-positive disease. The key to effective palliation is balancing the potential benefits with the expected side effects of specific therapies. Preclinical studies and some preliminary clinical data suggest that aromatase inhibitors are not likely to be effective and may be dangerous in premenopausal women. Hematology and Oncology:Question 103 The correct answer is D Educational Objectives Treat metastatic breast cancer. In the latter case. endocrine therapy is almost always less toxic than chemotherapy. resection of both lesions might even offer the possibility of cure. A randomized study showed a 1-year survival advantage for patients with a single metastasis to the brain treated with surgery and radiation therapy compared with radiation therapy alone. and the palliative benefits are well recognized. . have been consistently negative. A finding of bone metastasis would be best treated by palliative radiation therapy or chemotherapy or both. surgical therapy is considered in selected situations of isolated single metastasis. especially if the second lesion is not malignant. For many patients. if not synergistic. In this regard. Surgical therapy might also be called for to relieve an epidural compression if radiation therapy is contraindicated or if a second lung nodule is found that is suspected of being cancerous. A finding of liver metastasis or cytologically positive pleural effusion would be best treated by palliative chemotherapy. curative intent is most unlikely. she should be treated with therapy that has a higher chance of working.Hematology and Oncology:Question 102 The correct answer is C Educational Objectives Recognize the indications for surgical therapy after diagnostic testing for non-smallcell lung cancer.

This score is an extremely important prognostic factor in predicting the biology of clinically localized prostate cancer. with the highest scores predictive of patients who will not have organ-confined disease. many patients will commence long-term androgen ablation. Since the patient’s upper abdominal discomfort is vague and mild. best supportive care and referral to a hospice is the best recommendation. Hematology and OnCology:Question 105 The correct answer is A Educational Objectives Provide supportive care in the management of pancreatic cancer. This patient is not a good candidate for chemotherapy. This patient with a high Gleason score (despite the fact that his examination reveals no abnormalities and his PSA is low) would have only about a 37% probability of having organ-confined disease. a trial of narcotic analgesics is warranted. Critique It is important for this patient to realize that with long-term androgen ablation he will be at risk for osteopenia and potential nonpathologic fractures. The use of PSA screening has led to the earlier detection of prostate cancer recurrence after definitive local therapy. The Gleason score is obtained by grading from 1 to 5 the two predominant histologic types of adenocarcinoma of the prostate and adding the grades. There is some variability among pathologists when scoring prostate cancer using this system. Leuprolide treatment should not impair urinary flow in this asymptomatic patient. Diagnostic biopsy is not needed because it would not influence the current management. that the Gleason score can be used as a prognostic variable. Weight gain and loss of muscle mass can occur with testosterone . Even if the patients clinical status improves slightly as he recovers from the pulmonary embolism. Critique This patients Gleason score indicates that he would have a high risk of recurrence after prostatectomy. There is no standard therapy for these patients. Critique This patient almost certainly has metastatic pancreatic cancer. Palliative radiation therapy is indicated only for the relief of local symptoms. The scores range from 2 to 10. The concordance rate is high enough. therefore. Although there is no evidence that early hormone therapy can affect survival in this group. The median time to disease progression of patients with advanced pancreatic cancer-related symptoms is only a few months. his baseline performance status is poor because of his other medical conditions and cachexia caused by the cancer. he is unlikely to benefit from palliative chemotherapy.Hematology and Oncology:Question 104 The correct answer is C Educational Objectives Recognize the clinical significance of the Gleason score in patients with newly diagnosed prostate cancer. Many patients are asymptomatic and have no clinical manifestations of cancer except for an abnormal PSA level. The Gleason score is not influenced by the absence of symptoms in a patient. Hematology and Oncology:Question 106 The correct answer is B Educational Objectives Recognize the potential toxicities of androgen suppression. however. As such. even with the best available chemotherapy.

Furosemide must not be given until the patient has first been adequately rehydrated. Despite her history of congestive heart failure. focusing on sites that drain to the right inguinal region. many patients note loss of hair on their arms and legs with leuprolide therapy. In general. vimentin). Hematology and Oncology:Question 109 The correct answer is C Educational Objectives Select the best test to evaluate the histology of carcinoma of unknown primary site. A meta-analysis of randomized trials documents that combined-modality therapy is the preferred option. Approximately 25% of patients with limited-stage small-cell lung cancer will be free of disease 5 years after combined-modality therapy. primary sites might include the eye or even mucosal areas that can receive sun exposure. Critique Clinical staging for small-cell lung cancer classifies disease as limited-stage (unilateral lung and mediastinal involvement) or extensive-stage (bone. Critique The patient has hypercalcemia. Surgery is not indicated because of the systemic nature of small-cell lung cancer. If positive. in addition. with chemotherapy and radiation therapy administered concomitantly at the start of treatment. Critique When a poorly differentiated cancer is negative for leukocyte common antigen and cytokeratin. Although corticosteroids can be considered as an adjunctive therapy in a patient with a potentially hormoneresponsive tumor such as breast cancer. Hematology and Oncology:Question 107 The correct answer is D Educational Objectives Treat limited-stage small-cell lung cancer. liver. it would slow the necessary decrease in her serum calcium level. the most common metabolic complication of malignancy.suppression. The best test for establishing melanoma is a stain for S-100. An inguinal lymph node would be an expected site for melanoma but very unusual for sarcoma. the top priority in this seriously dehydrated patient is volume repletion. the most appropriate initial therapy is to rehydrate as vigorously as possible with normal saline. In addition. Sometimes the primary site may not be evident. although other immunoperoxidase stains can be employed (melan A. the most likely diagnosis is melanoma (which can be amelanotic) or sarcoma. even when the tumor is known to be a melanoma. Administration of a bisphosphonate is not an appropriate initial therapeutic intervention in this patient because of her volume depletion. brain. She is profoundly dehydrated. or bilateral lung metastases). Melanoma can be further confirmed with electron microscopy. . an exhaustive search of the skin should be undertaken to locate the primary tumor. Hematology and Oncology:Question 108 The correct answer is C Educational Objectives Treat symptomatic hypercalcemia of malignancy.

ventilation/perfusion scan is not useful to establish a diagnosis in SVC syndrome Hematology and Oncology:Question 111 The correct answer is B Educational Objectives Treat metastatic testicular carcinoma with chemotherapy. Critique Her family should be reassured that they are not at particularly high risk for cancer. etoposide. whose tumor has recurred in the lungs (a common location after retroperitoneal lymph node dissection). and cisplatin. A contrast-enhanced CT is the most useful test to diagnose SVC syndrome and to detect the presence of a mediastinal mass. and external compression of mediastinal venous channels. Teratoma is not chemoresponsive. and both she and her family should be encouraged to . generally after treatment with chemotherapy if there are remaining nodules. not pulmonary embolism. Because the patient does not have any life-threatening symptoms such as upper airway obstruction. There is no advantage to watchful waiting in a potentially curable patient. and. A venous dye study would not add to the findings of the CT. even for patients with advanced disease. Radiation is sometimes used for palliation. Mediastinoscopy with biopsy is more invasive than necessary for this diagnosis. Critique The presentation is consistent with obstruction of the superior vena cava (SVC). after chemotherapy. Hematology and Oncology:Question 112 The correct answer is D Educational Objectives Recommend appropriate cancer risk assessment and testing. this patient. should be treated with curative intent. Therefore. it may also provide an opportunity for CT-guided needle biopsy. Surgical resection of lung lesions is sometimes appropriate in testicular cancer. The clinical picture does not suggest pulmonary embolism. decreased opacification of the innominate vein or SVC below the level of obstruction.Hematology and Oncology:Question 110 The correct answer is B Educational Objectives Recognize the presentation of superior vena cava syndrome and identify the most useful initial diagnostic test. CT findings indicating SVC obstruction may include intraluminal filling defects of the SVC. Critique Testicular carcinoma is the rare metastatic solid tumor that can be routinely cured with combination chemotherapy such as bleomycin. but chemotherapy would be the appropriate choice for multiple asymptomatic lesions. Initiating both pulmonary and thoracic surgery consultations early on is prudent to facilitate the work-up and obtain a tissue diagnosis. residual tumor of this histologic type may need to be resected. opacification of collateral vessels. he should undergo diagnostic procedures to confirm the cause and guide the most appropriate therapy. The introduction of cisplatin approximately 25 years ago has resulted in effective therapy. Administration of radiation therapy while the diagnostic work-up is occurring is not necessary because the patient’s symptoms do not require emergency intervention. especially the younger ones.

Pain management is an important part of the care of many cancer patients. Critique The only one of the options that is contraindicated is intravenous gamma globulin. . Testing is very expensive and can be associated with occasional false-positive results. as one might see in families that harbor BRCA-1 or BRCA-2 (breast and ovarian cancers). Patients with multiple myeloma have hypogammaglobulinemia when the paraprotein is not considered and are susceptible to infections. it can be used in patients who have recurrent episodes of bacterial infections or sepsis that are life-threatening. and randomized studies have found that bisphosphonate therapy improves patients’ length and quality of life. and they often result in multiple cancers in the same person. has not been shown to reduce the incidence of serious infections when used prophylactically. Radiation therapy may be necessary to control localized. Erythropoietin has been shown to alleviate the anemia and improve quality of life. these data suggest that the cancers observed in this family are sporadic and it is very unlikely that her family harbors a germ-line abnormality in any of the currently identified tumor suppressor genes. Even though there are two instances of breast cancer in the family. Intravenous gamma globulin.follow routine screening and risk reduction guidelines. Hematology and Oncology:Question 113 The correct answer is C Educational Objectives Apply the principles of supportive care in multiple myeloma. however. Although several members of this patients family have had cancer. Taken together. there is no specific pattern of organ-oriented cancers. Evidence is also emerging for a role for bisphosphonates in killing tumor cells. Furthermore. The goal of total relief of pain should not be sacrificed to fears of causing narcotic addiction in this population. Furthermore. However. symptomatic bony destructive lesions. They also control hypercalcemia. Monthly intravenous gamma globulin is not routinely administered to patients with myeloma. Hereditary genetic cancer syndromes are usually associated with cancers in patients younger than 60 years. Bisphosphonates such as pamidronate or zoledronate are effective agents at minimizing bony destruction and bone fractures. the patients ancestry is not one in which hereditary genetic cancer syndromes are more prevalent. both occurred in elderly women. The supportive care of the patient with myeloma is as important as the anticancer treatment to the patients quality of life. which could have devastating consequences for her or members of her family. in each case the cancer was one that occurs commonly in the age groups in which these individuals were diagnosed.

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