Anemia o Reduction in EPO Activity o Clue Ferritin High o Transferrin Receptors Low Or Normal o Decreased reticulocyte count Microcytic Anemia • Iron Deficiency Anemia Most Common Cause Always Rule out first o RBCs becomes heterogeneous in size and shape ↑ RDW o Result in 2nd Thrombocytosis less than a million o Consider Cancer in Elderly with +Ve FOBT o Changes in Order ▪ Labs > Hgb > Morphologies o Tx Oral iron, IV for those who can’t absorb Oral Iron (celiac) ▪ Hgb Concentration will rise by 2 gm\dl in 1-2 weeks after therapy o A Serum ferritin level >100 ng/mL rules out iron deficiency. • Lead Poisoning Sideroblastic RBC • Thalassemia o Alpha Thalassemia 4 Alpha Chains ▪ Silent Carrier,1 Deletion ▪ Trait, 2 Deletion, Mild microcytic anemia Normal hemoglobin electrophoresis Clue Normal RDW ▪ Hb H, 3 Deletion Tx Frequent Transfusion o Beta Thalassemia 2 Beta Chains ▪ Trait 1 Deletion Normal RDW, Elevated hemoglobin A2 in electrophoresis. ▪ Major 2 Deletion TX Frequent Transfusion, Macrocytic • Megablastic Anemia MCV >115 a trilineage Disorder Anemia o B12 ▪ Seen in Alcoholic and IF Deficiency (Pernicious Anemia) and Metformin users ▪ Confirmed with MVA and Homocysteine Elevated ▪ Copper Deficiency is a mimicker o B9 ▪ Only Homocysteine Elevated o Fanconi anemia may cause Aplastic Anemia ▪ Primary • DNA repair defect causing bone marrow failure macrocytosis may be seen on CBC • short stature, cafe-au-lai t spots with thumb/radial defects. ▪ Secondary to • AG, Heavy Metal Poisoning, Valproic Acid Hemolytic Anemia • Congenital hemolytic anemias o Sickle Cell Disease Low MCV ▪ AR Disorder ▪ Most Common organism for osteomyelitis S.Aurues but they could have Salmonella . ▪ The Could Develop AVN of the Hip ▪ Aplastic Anemia with B19 Infection Rash and Pancytopenia ▪ In VOC hydration is a must with good pain control
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Hematology SBIM Part 1 High Yield Facts
▪ Hydroxyurea Increase Hgb Fetal
• Indication More than two pain crises each year or for those with ACS • Response is assessed by ↑ MCV • Decrease Mortality ▪ Exchange transfusion is indicated for patients with an acute stroke, fat embolism, or ACS. • ACS is usually characterized by pulmonary infiltrates, fever, chest pain, tachypnea, and hypoxemia o G6PD X-linked ▪ Drug Induced and fava beans ▪ Normal Enzyme Level in Crisis o Spherocytes ▪ Hereditary DAT +Ve ▪ Acquired DAT -Ve • Alcoholism • Hemolytic Transfusion reaction o Sideroblastic ▪ Acquired B6 or Cooper Deficiency, INH or Alcohol usage. • Acquired hemolytic anemias o Drug Induced Quinine, Penicillin, α-Methyldopa o Autoimmune Hemolytic Anemia ▪ Warm autoimmune hemolytic anemia IgG • CLL, NHL and SLE • Tx Glucocorticoids+/- splenectomy > IVIG > Rituximab ▪ Cold agglutinin disease IgM • Seen in Mycoplasma infection and Plasma Disorder • Cold avoidance or rituximab for persistent symptoms • Glucocorticoids or splenectomy are usually ineffective. Neutropenia • Less than 1000 • Acute HIV, CMV, EBV • B12, Copper and Zinc Deficiency • Cytotoxic chemotherapies • NSAIDs, carbamazepine, phenytoin, propylthiouracil, cephalosporins, trimethoprim-sulfamethoxazole and Ganciclovir • SLE, RA (Felty Syndrome) • Tx o Remove the offending drug. o Granulocyte colony-stimulating factor if neutropenia is complicated by infection Eosinophilia • Eosinophil counts greater than 1500/μL • Connective tissue diseases, like EGPA and RA • Helminths aka parasites • Idiopathic, Hyper eosinophilic syndrome
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Hematology SBIM Part 1 High Yield Facts
• Neoplasms Hodgkin lymphoma and CML
• Allergies asthma and drug-induced eosinophilia, DRESS and Addison’s disease, for some reason.
Bleeding Disorders • Mixing Study
o NOT Corrected Antibodies against coagulation factors o Corrected Deficiency coagulation factors • Prolonged Bleeding time a Platelet Disorder o Acquired ▪ Scurvy ▪ Uremia ▪ MDS Hemophilia • X-linked von Willebrand • Type 1 (AD) Quantitative, Type 2 Qualitative, Type 3 (AR)Sever Disease Quantitative like Hemophilia. • Most Common Inherited Disorder • Mucocutaneous Bleeding • Prolonged BT and a normal or prolonged a PTT • Secondary hemostatic dysfunction can occur due Low Factor 8 • Heyde's syndrome (AS) acquired von Willebrand Disease • Treatment o Mild, OCP or Tranexamic Acid to Control bleeding o Moderate or Before Simple procedure, DDAVP o Sever, Factor 8 Concentrate Thrombocytopenia • Important Values for Platelet transfusion o Bleeding or planned surgery 50,000/μL o No other risk factors for bleeding 10,000-20,000/μL o Platelet transfusion for intracranial bleeding 100,000/μL • Immune thrombocytopenia o Idiopathic immune thrombocytopenia o Drug-induced ITP Commonly Antibiotics o Disease-associated immune thrombocytopenia HIV, HBV, Thyroid Disease and malignancy ▪ Indication for treatment • Evident Bleeding • Plt. Count less than 30 K ▪ Treatment • Glucocorticoids • IVIG • Last Resort Splenectomy or rituximab • Consumptive Thrombocytopenia o TTP a Hematological Emergency DON’T WAIT Treat with plasma exchange. ▪ Thrombocytopenia and Hemolytic anemia (Clue) ▪ Plus, Fever, Mental State changes and AKI ▪ ADAMST13 Deficiency resulting large multimers of vWF in their plasma ▪ Can be Acquired with Quinine, Clopidogrel, Cyclosporine or Chemotherapeutic agent.
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Hematology SBIM Part 1 High Yield Facts
▪ Lab Shows Elevated LDH and Bilirubin with
fragmented RBC. ▪ Normal PT, aPTT, D-dimer, and fibrinogen levels o DIC ▪ Thrombocytopenia and schistocytes ▪ Abnormal PT, aPTT, D-dimer, and fibrinogen levels o HELLP ▪ Hemolysis, Elevated liver enzymes, and a Low platelet count ▪ Occurs in association with pre-eclampsia or eclampsia ▪ Treatment Delivery o HUS ▪ Escherichia coli O157:H7 or Shigella infections ▪ Generally, within 6 days after diarrhea onset Heparin-Induced • Type 1 Direct Destruction Thrombocytopenia • Type 2 Antibodies mediated Thrombosis and Thrombocytopenia and Thrombosis o Platelet decrease of >50% in a patient taking heparin o or a thromboembolic event 5 to 10 days after starting heparin o Tx Stop Heparin and start argatroban, fondaparinux or bivalirudin to treat Thrombosis Thrombophilia • Acquired Thrombophilia o Antiphospholipid antibody syndrome ▪ Lupus anticoagulants prolong PTT Confirmation Russel viper venom associated with thrombosis ▪ Anticardiolipin antibody ▪ Diagnosed with Abs Positive in 2 occasions 12 weeks apart. ▪ Recurrent Miscarriage at least 3 before 10th week or one death after 34th weeks ▪ Tx Long term OAC • Inherited Thrombophilia o Factor V Leiden mutation Homozygous mutation is an indication for prolonged OAC o Antithrombin III can be lost in Nephrotic Syndrome ▪ They become heparin Resistant Transfusion • An Acute hemolytic transfusion reaction Medicine o ABO incompatibility o Positive DAT o Hypotension and Tachycardia • A Delayed hemolytic transfusion reaction High Yield o Delayed emergence of an alloantibody 2 to 10 days after transfusion o Extravascular hemolysis elevated serum bilirubin and LDH levels new alloantibody • A Febrile nonhemolytic transfusion reaction o Donor leukocyte or alloantibodies directed against donor leukocytes o Tx Antipyretics • Transfusion-related acute lung injury
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Hematology SBIM Part 1 High Yield Facts
o ARDS (hypoxemia and noncardiogenic pulmonary edema)
o Leukocyte aggregation in the pulmonary capillary bed within 6 hours • Transfusion-associated circulatory overload o Most common therefore we give Lasix in susceptible individuals. o Within 6 hours of transfusion • An allergic transfusion reaction o Especially in IgA deficiency ▪ Recurrent Sinopulmonary Disease ▪ Associated with autoimmune disease Tumor lysis • UA level need to be Drawn on Ice pack Syndrome • Labs ↑K+ ↑UA ↑ PO4 ↓Ca2+ • May be the first manifestation of AML • Management o Allopurinol and hydration Increase UOP for Moderate UA level o Rasburicase for Sever UA level or aggressive tumor Acute Myeloid • Proliferation of myeloid cells with Auer rods Leukemia • Leukostasis syndrome leukocyte count is very high CNS sx and Lung sx o Leukapheresis if leukocyte count >50 K or With Symptoms • Labs ↑↑↑ Leukocyte count, Anemia, thrombocytopenia, and Blasts • Definitive Diagnosis Bone marrow biopsy showing >20% Blasts. • Pancytopenia, but bone marrow will be hypercellular with 20% or more blasts • favorable risk: t(8;21), inv(16), t(15;17) o t(15;17) Can Develop DIC treatment ATRA Vit A Analogue ▪ ATRA Side Effect; Differentiation syndrome ▪ Fever, pulmonary infiltrates, hypoxemia, and, occasionally, hyperleukocytosis. ▪ Treatment is Dexamethasone
Acute • Extremely Aggressive
Lymphoblastic • CNS involvement Leukemia • CNS prophylaxis (intrathecal chemotherapy with or without radiation) Because standard chemotherapy cannot get into the CNS and kill cancer cells Chronic Myeloid • Confirmed by the presence of the Philadelphia chromosome Leukemia (activated tyrosine kinase) o Bad Prognosis o Treatment Tyrosine kinase inhibitors: imatinib • Elevated leukocyte count Basophils are Your Clue Almula Rule Chronic • Asymptomatic Lymphocytic • Diagnosis is confirmed by flow cytometry demonstrating cell surface Leukemia antigens CD5 and CD23. • Smudge cells • Low serum IgG levels require replacement therapy to prevent infection.
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Hematology SBIM Part 1 High Yield Facts
Leukemoid • Benign State
Reaction • High leukocyte alkaline phosphatase • Response to stressors (eg, infections, medications, severe hemorrhage) Lymphoma • Enlarging lymphadenopathy associated with systemic symptoms • To diagnosis lymphoma an excisional biopsy is a must then follow it with a full body imaging with PET for Staging o Hodgkin Lymphoma >>> Reed-Sternberg cells ▪ Aggressive lymphomas require immediate therapy and often can be cured. ▪ Staging Spleen is not part of the staging • Stage I one lymph node region or organ. • Stage II Two or more lymph nodes are affected. Above or below the diaphragm. • Stage III Both sides of the diaphragm. • Stage IV Every Where ▪ Treatment: ABVD followed by radiation therapy • Most Questions are about complications • (A)driamycin aka doxorubicin > Dilated CMP • (B)leomycin > Lung Fibrosis • (V)inblastine > Peripheral neuropathy • (D)acarbazine > Sterility ▪ Radiotherapy increase Risk for secondary cancers (breast, lung, skin) and CAD. o Non-Hodgkin lymphoma Plasma Cell • Multiple Myeloma Disorder o Signs and symptoms ▪ C (hyperCalcemia) ▪ R (Renal failure) ▪ A (Anemia) ▪ B (Bone disease: lytic lesions, fractures, or osteoporosis) o Testing SPEP or UPEP a clue is low anion gap o a Skeletal Survey to assesses for lytic bone lesions Do not use bone scans. o Even if you suspect MM but the presentation is an cute neurological manifestation MRI Spine is your answer • Waldenström Macroglobulinemia o A Fake ☺ Lymphoma (Lymphadenopathy, hepatomegaly, and splenomegaly) o IgM Elevated (Pentamer) o Hyperviscosity syndrome an emergency treated with plasmapheresis.
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Hematology SBIM Part 1 High Yield Facts
Myeloproliferative • Polycythemia Vera
Neoplasms o Hemoglobin level is >16.5 g/dL in men or >16 g/dL o Low EPO level a clue to rule out secondary causes (hypoxemia, kidney or liver carcinoma and use of androgens) o The JAK2 mutation 97% o Budd Chiari and Hepatic vein thrombosis r\o PV o Pruritus (↑ hot water), and SPLENOMEGALY o LOW DOSE ASA is Indicated (High Dose result in Thrombosis) o Treatment ▪ Low Risk • Phlebotomy Hct level to <45%. ▪ High Risk Group • Old Above 60 yrs • History of thrombosis • Elevated WBC count • Phlebotomy and Hydroxyurea • Essential Thrombocythemia o Platelet counts greater than 450,000/μL o The JAK2 mutation 50% o Hyper Viscosity Symptoms o Treatment ▪ Low Risk • ASA to reduces Hyper Viscosity ▪ High Risk Group • Old Above 60 yrs • History of thrombosis • Elevated WBC count above 11K • ASA and Hydroxyurea ▪ Plateletpheresis in life-threatening situations or to reduce count rapidly • TIA, stroke, MI, or GI bleeding • Myelofibrosis o Teardrop erythrocytes, and bone marrow fibrosis o Splenomegaly and hepatomegaly result from extramedullary hematopoiesis. o Death transformation to acute leukemia, or portal hypertension complications