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Hematology SBIM Part 1 High Yield Facts

Normocytic • Inflammatory Anemia


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

Alkhazmari Legacy Notes 1


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

Alkhazmari Legacy Notes 2


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.

Alkhazmari Legacy Notes 3


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

Alkhazmari Legacy Notes 4


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.

Alkhazmari Legacy Notes 5


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.

Alkhazmari Legacy Notes 6


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

Alkhazmari Legacy Notes 7

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