Card Set Information

2013-10-30 21:48:44
medicine shelf

hematology - MKSAP, USMLE
Show Answers:

  1. pruritis while showering
    elevated hematocrit
    elevated platelet count
    polycythemia vera
  2. Polycythemia vera
    • Dx: elevated RBC mass, normal arterial oxygen saturation, splenomegally
    • Morbidity: thrombosis (risk reduced when hematocrit <45%)
    • Tx: phlebotomy
  3. Myelodysplastic syndrome
    • cytopenia of at least one lineage
    • often more than one lineage
  4. Anemia of inflammation (chronic disease)
    • iron levels decrease: sequestration of iron in body storage pools 2/2 increase hepcidin and binding of iron by bacterial lactoferrin
    • low total iron-binding capacity
    • low transferrin levels
    • normal to high ferritin levels
  5. Iron-deficiency anemia
    • decreased ferritin levels
    • low serum iron
    • High TIBC
  6. Sideroblastic anemia
    • high ferritin
    • low serum iron
    • low TIBC
  7. Thalassemia
    • Normal ferritin
    • Normal serum iron
    • Normal TIBC
  8. pancytopenia
    hypocellular bone marrow
    • Aplastic anemia
    • Cause: 50% idiopathic
    • Tx: immunosuppressive therapy (antithymocyte globulin and cyclosporine) reduces transfusion requirements
  9. anemia
    elevated reticulocyte count
    microspherocytes on peripheral blood smear
    • Autoimmune hemolytic anemia
    • Most common type: warm-antibody mediated
    • -IgG antibodies bind the Rh-type antigens at 37 degrees C --> erythrocyte destruction by splenic macrophages
    • Dx: direct Coombs test
  10. Thrombotic thrombocytopenic purpura (TTP)
    • presentation: neurologic symptoms
    • fragmentation anemia
    • thrombocytopenia
    • microangiopathic hemolytic anemia: fragmented and nucleated red blood cells; low platelet counts
  11. Macrocytic anemia
    elevated lactate dehydrogenase
    elevated unconjugated (direct) bilirubin levels
    elevated methylmalonic acid (MMA)
    elevated homocysteine concentration
    • Vitamin B12 deficiency
    • associated with neurologic findings, forgetfulness
    • Serum B12 is NOT a good measurement of tissue cobalamin levels
  12. folate deficiency
    elevated homocysteine concentration only
  13. spherocytic RBC
    elevated mean corpuscular hemoglobin concentration
    • hereditary spherocytosis:
    • disorder of the skeletal membrane proteins in RBCs
    • mutation in the spectrin gene, ankyrin gene, Band 3 or Band 4.2
  14. hypersplenism
    • can induce hemolysis
    • decrease the number of leukocytes, platelets, or any combination of cell lines
  15. microcytic, hypochromic RBC
    anisocytosis (abnormalities in size)
    poikilocytosis (abnormalities in shape)
    iron deficiency anemia
  16. microcytic anemia
    prominent target cells
    • alpha-thalassemia
    • pts have preserved or increased red blood cell counts compared with patients with iron deficiency
  17. iron deficiency anemia
    older than 50
    must rule out colon cancer: colonoscopy
  18. G6PD deficiency
    • acute hemolysis after ingesting primaquine or sulfa drugs
    • most common enzymatic disorder of RBCs in human
    • X-linked disorder
    • G6PD levels often normal during the hemolytic episodes
    • Bite cells
  19. Lung cancer in non-smokers
    • adenocarcinoma of lung has the least association with smoking
    • most common type of lung cancer
    • location: peripherally, consists of columnar cells growing along the septa
  20. Vitamin K deficiency
    • important co-factor for hemostasis
    • Risk factors: NPO, broadspectrum antibiotics
    • Stores: 30 days in a normal liver; 7-10d in an acutely ill patient
    • Factors: 2, 7, 9, 10, protein C and S (prolonged PT and PTT
  21. Single pulmonary nodule
    • 3cm or less, surrounded by normal parenchyma
    • most are benign
    • calcification favors a benign lesion
    • popcorn calcification is a hamartoma
    • bulls eye is a granuloma
  22. Paroxysmal nocturnal hemoglobinuria (PNH)
    • cell membrane anchor protein GP1 abnormality
    • normally inhibits destruction of RBCs
    • intravascular hemolytic anemia
    • Labs: elevated bilirubin, LDH
    • hepatic vein thrombosis
    • Dx: flow cytometry for presence of CD55 and CD59 protein
  23. Macrocytic anemia
    common causes
    • Folate deficiency
    • Vitamin B12 deficiency
    • Myelodysplastic syndromes
    • Acute myeloid leukemias
    • Drug-induced (hydroxyurea, zidovudine, chemotherapy agnets)
    • Liver disease
    • Alcohol abuse
    • Hypothyroidism
  24. Pernicious anemia
    • Most common cause of vit B12 deficiency in whites of northern European background
    • commonly have other associated autoimmune diseases (autoimmune thyroid disease vitiligo)
    • Pex: shiny tongue (atrophic glossitis), ataxia, neurologic abnormalities (position vibratory sense)
  25. Beta thalassemia
    • usually asymptomatic with mild anemia, disproportionaetly high RBC count
    • low MCV
    • hemoglobin >10
    • often confused with iron deficiency anemia
    • Tx: no specific therapy
  26. lupus anticoagulant
    • occurs in 5% of SLE patients
    • VTE
    • labs: prolongs PTT by binding the phospholipids in the assay (artifactual)
    • PTT will NOT correct if mixed in a 1:1 dilution
    • Russell viper venom test: prolonged in the disease
  27. Lupus anticoagulant
    • 1. PTT will be normal or slightly prolonged
    • 2. VWf, bleeding time, platelet count will be normal
    • 3. D-dimer will be normal or hight
  28. Sickle cell
    stroke treatment
    • Focal deficit, think stroke
    • even if CT is negative (in the first few hours)
    • Tx: Exchange transfusion (prevents a second infarct from occurring)
  29. Waldenstrom's macroglobulinemia
    • rare, chronic, plasma cell neoplasm: IgM spike and hyperviscosity
    • 1. increased size of the spleen, liver, and some LNs
    • 2. Tiredness, usually due to anemia (too few RBCs)
    • 3. Tendency to bleed and bruise easily
    • 4. Night sweats
    • 5. Headache and dizziness
    • 6. Various visual problems
    • 7. Pain and numbness in the extremities due to a predominantly demyelinating sensorimotor neuropathy

    Hyper-production of IgM antibody in the blood
  30. Tumor lysis labs
    • Calcium: Decreased
    • Phosphate: Increased
    • Potassium: increased
    • Uric acid: increased
  31. CML
    • chronic myeloid leukemia
    • philadelphia chromosome: abnl chrom22 translocation (9-22) forming the BCR-ABL fusion gene 
    • abnormal tyrosine kinase activity
    • Tx: tyrosine kinase inhibitors (Imatinib Gleevec) that bind to the ATP binding site of the BCR-ABL protein, prohibiting the conformation change to its active form. Non-receptor tyrosine kinase
  32. Thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS)
    • Fragmented cells in the peripheral smear: MAHA
    • Presentation: hemolytic anemia and thrombocytopenia, renal failure and neurologic symptoms
  33. TTP-HUS
    • deficiency of or autoantibody against a specific von Willebrand factor cleaving protease (ADAMTS-13)
    • accumulation of large vWf multimers and platelet aggregation
    • Tx: plasmapheresis (removes offending autoantibodies) otherwise fatal in 80% of cases
  34. Prostate cancer
    • adrogen ablation (orchiectomy)
    • if recurrent, radiation therapy
  35. Multiple myeloma
    • monoclonal proliferation of plasma cells
    • CRAB: calcemia, renal impairment, anemia, bones (bone pain, lytic lesions, fractures)
    • IgG antibodies, or paraproteins, produced by the myeloma cells can collect in glomeruli, causing renal failure, "myeloma kidney"
    • -risk for infection: decrease in functional antibodies and leukopenia
  36. MALT
    low grade
    • cause: H pylori
    • Tx: triple therapy (omeprazole, clrithromycin, amoxicillin)
    • if fails: chemotherapy (CHOP)
  37. CHOP
    • Cyclophosphamide
    • adriamycin
    • vincristine
    • prednisone
  38. Anemia of chronic disease
    • normocytic anemia
    • decreased serum iron concentration
    • decreased TIBC (vs. iron deficiency anemia where TIBC is elevated)
    • mild decreased transferrin saturation (usually 15-20%)
    • Ferritin level: normal or high
    • pathophys: iron trapping within macrophages
  39. Treatment of RA
    • methotrexate
    • hydroxychloroquine
    • TNF inhibitors (infliximab, etanercept)
  40. Tamoxifen
    • endometrial cancer
    • uterine sarcoma
  41. Syphilis testing
    • VDRL is highly sensitive, but not specific
    • must confirm with FTA-ABS test
    • False positives: Antiphospholipid antibody syndrome (APS)
  42. APS
    antiphospholipid antibody syndrome
    • Can promote arterial and venous thromboses
    • tendency toward spontaneous abortions
    • Tx: low molecular weight heparin (in pregnancy)
  43. Cushing syndrome
    • Clinical manifestations:
    • -progressive central obesity
    • -skin atrophy, wide puplish striae
    • -proximal muscle weakness
    • -hypertension
    • -glucose intolerance
    • -skin hyperpigmentation

    • Dx:
    • -24-hr urinary cortisol excretion
    • -late evening salivary cortisol
    • -low-dose dexamethasone suppression test
  44. warm-antibody autoimmune hemolitic anemia
    • polychromasia from reticulocytosis
    • IgG antibodies bind RBC antigens (Rh-type) at body temperature
    • cleared by splenic macrophages
    • Tx: corticosteroids
  45. vWD
    • suspected if aPTT, bleeding time, or PFA-100 is prolonged
    • associated with factor VIII deficiency
    • primary hemostatic dysfunction: mucosal bleeding, menorrhagia, purpura
  46. Hemophilia A and B
    • X-linked recessive disorders
    • joint and deep muscle bleeding
    • Deficiency of factor VIII and IX, respectively
  47. Thrombotic thrombocytopenic purpura
    • thrombocytopenia, fever, MAHA, acute neurologic symptoms
    • MAHA: elevated lactate dehydrogenase, anemia, schistocytes
    • renal disease
    • Tx: plasma exchange (urgently)
  48. Hemolytic uremic syndrome
    thrombocytopenia, MAHA, renal disease
  49. Heparin-induced thrombocytopenia (HIT)
    • rapidly falling platelet count shortly after initiation of heparin
    • tx: immediately stop heparin, switch to direct thrombin inhibitor
  50. Immune thrombocytopenic purpura
    • dx of exclusion
    • severe thrombocytopenia in patient with normal hemoglobin level and a normal leukocyte count
    • absence of fever, headache, constitutional symptoms
  51. VTE risk
    • estrogen-containing oral contraceptives
    • risk is increased 35-fold in women who are heterozygous for factor V Leiden mutation (anti-thrombin)
  52. Factor V Leiden, prothrombin G20210A
    • most common mutations predisposing to VTE in white populations
    • rare in Asian and black
  53. Phyladelphia chromosome
    • Chronic myeloid leukemia
    • Tx: Imatinib mesylate (targets and inhibits BCR-ABL tyrosine kinase activity)
    • complete remission in 95% of patients vs 56% of those treated with interferon and low-dose cytarabine
  54. Chronic lymphoid leukemia
    • most common leukemia encountered in adults
    • fever, night sweats, weight loss, fatigue, malaise
  55. Splenomegaly sx
    • early satiety
    • left upper quadrant pain
  56. Acute leukemia
    • >20% blasts
    • Tx: induction chemo with cytarabine and an anthracycline
  57. multiple myeloma
    • hypercalcemia, bone pain, anemia
    • bone marrow biopsy shows large plama cells
  58. MGUS
    • monoclonal gammopathy of unknown significance
    • characteristics: serum monoclonal gamma-globulin without clinical features of multiple myeloma, low paraprotein level, <10% plasmacytosis in the bone marrow
    • Tx: repeat serum protein electrophoresis in 3 to 6 months