Heme step2

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gm1147
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165872
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Heme step2
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2012-08-13 17:35:46
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Hematology step2 heme
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  1. Symptoms of anemia at hct
    • 25-30%: dyspnea, fatigue
    • 20-25%: lightheaded, angina
    • <20%: syncope, CP
  2. Microcytosis causes, other findings
    • Fe deficiency
    • Thalassemia
    • Sideroblastic
    • AoCD
    • -Low retic, hypochromatic, target cells
  3. Macrocytic anemia causes, other findings
    • B12 def
    • Folate def
    • Alcoholism
    • Sideroblastic
    • Antimetabolite drugs (azathioprine, 6-mercaptopurine, hydroxyurea)
    • Zidovudine
    • Phenytoin
    • Liver disease
    • Hypothyroid
    • MDS
    • -Low retic counts
  4. Cryoprecipitate use
    • Replaces fibrinogen in addition to CF
    • Isnt first line
  5. Sideroblastic anemia
    • Micro or macrocytic
    • MDS, etoh, lead, isoniazid, Vit B6 def
  6. Studies in microcytic anemia:
    low Ferritin
    high Fe
    Normal study
    Inc RDW
    Prussian blue
    • Low ferritin = Fe def
    • High Fe = Sideroblastic
    • Normal Fe studies = thalassemia
    • Inc RDW = Fe def
    • Prussian = sideroblastic
  7. Fe def vs AoCD
    • Fe def: low Fe, low ferritin, inc TIBC
    • AoCD: low Fe, inc ferritin, low TIBC
  8. Alpha Thalassemia types
    • Alpha:
    • 2 deletions - mild anemia, normal electrophoresis
    • 3 - moderate anemia, HbH (beta tetrads), inc retics
    • 4 - gamma tetrads or HbBart, CHF and death in utero
  9. Causes of B12 def
    • Pernicious anemia
    • Pancreatic insufficiency
    • Dietary
    • Crohns
    • Celiac
    • Blind loop syndrome
    • D latum
  10. Folate deficiency causes
    • Dietary - none in goat milk
    • Psoriasis, skin loss/turnover
    • Phenytoin
    • Sulfa - MTX
    • Celiac
  11. B12 and folate def lab tests
    • Megaloblastic anemia
    • Inc LDH
    • Inc indirect bili
    • Dec retic
    • Hypercellular bone marrow
    • Macroovalocytes
    • Inc homocysteine
    • Can have pancytopenia
    • B12: inc methylmalonic acid
  12. Side effect of B12 or folate replacement
    Hypokalemia
  13. Dactylitis
    • Inflammation of fingers
    • Seen in children with sickle cell
  14. AS disease
    • Sickle cell trait
    • Smear is normal, do electrophoresis
    • Defect in urine concetration, can have hematuria
    • no tx
  15. Smear findings:
    Bite cells
    Schistocytes
    Morulae
    • G6PD def
    • Intravascular hemolysis
    • inside neutrophils in Ehrlichia infection
  16. Sickle cell tx
    • O2
    • Hydration
    • Analgesia
    • F or WBC: ceftriaxone, levofloxacin, or moxifloxacin
    • Folic acid replacement
    • Pneumococcal vaccine
    • Hydroxyurea
    • If severe - exchange transfusion
  17. Parvovirus in sickle cell management
    • initial: retic count - drop is first sign
    • IgM
    • accurate: PCR for DNA
    • Initial tx: IVIg
  18. Spherocytosis labs
    • low MCV
    • Inc MCHC
    • Neg Coombs
    • Accurate: osmotic fragility
  19. Warm hemolysis
    mech
    cause
    dx
    tx
    • Autoimmune, IgG
    • idiopathic, CLL, lymphoma, SLE, pen, rifampin, phenytoin
    • Accurate: Coombs
    • Tx: initial is steroids, splenectomy, IVIG, Rituximab
  20. Steroid weaners
    • Cyclophosphamide
    • Cyclosporine
    • Azathioprine
    • Mycophenolate mofetil
  21. Cold agglutin disease
    mech
    causes
    dx
    tx
    • IgM
    • EBV, Waldenstrom macroglobulinemia, M pneumoniae
    • direct Coombs - complement
    • Warm, rituximab, cyclophosphamide or cyclosporine. Not steroids or splenectomy
  22. G6PD def triggers
    • infection
    • Dapsone
    • Quinidine
    • Sulfa drugs
    • Primaquine
    • Nitrofurantoin
    • Fava beans
  23. G6PD tests
    • Initial: smear for Heinz and bite cells
    • accurate: G6PD level after waiting 1-2 mo (normal during hemolysis)
  24. HUS vs TTP
    • Both have intravascular hemolysis (leading to schistocytes), thrombocytopenia, and renal insufficiency
    • HUS is kids, TTP is adults
    • Different causes
    • TTP also has neurologic symptoms (AMS, sz) and fever
    • Both normal PT, PTT, Coombs
    • Tx with plasmapheresis or exchange
  25. TTP causes
    • ticlopidine
    • clopidogrel
    • cyclosporine
    • AIDS
  26. Paroxysmal Nocturnal Hemoglobinuria mechanism
    • Defective PIG-A gene
    • Overactivation of complement system
    • Deficiency of complement regulatory proteins CD55 and 59 (decay accelerating factors)
    • Increased sensitivity of RBC to complement in acidosis
    • Sleep leads to hypoventilation - mild acidosis - hemolysis and thombosis
    • aplastic anemia, myelodysplasia, or acute leukemia
  27. PNH dx
    • inital: CBC with pancytopenia
    • Accurate: CD55 and 59 levels/flow cytometry
    • Ham test or Sucrose hemoylsis to activate complement
  28. PNH tx
    • Prednisone
    • Allogenic bone marrow transplant for cure
    • Eculizumab inactivates C5
    • Folic acid
    • Transfusions
  29. Aplastic anemia causes
    • Infection: HIV, hep, CMV, EBC
    • Cancer
    • Radiation
    • Toluene, DDT, benzene
    • Drugs: sulfa, phenytoin, carbamazepine, chloramphenicol, etoh, chemo, PTU, methimazole
    • SLE
    • PNH
    • B12 or folate def
  30. Aplastic anemia tx
    • Cause
    • Bone marrow transplant
    • If >50 or no donor, antithymocyte globulin and cyclosporine/tacrolimus to inh T cells attacking other cells
  31. Polycythemia vera mech, presentation
    • mutation in JAK2 leads red cells (and all others) grow wildly
    • Hyperviscosity leads to HA, blurred vision, tinnitus, HTN, fatigue, splenomegaly, bleeding, thrombosis, pruritis after warm showers
  32. P vera dx and tx
    • Hct >60%
    • Inc plt and WBC
    • Total red cell mass inc
    • O2 normal
    • Epo low
    • B12 inc
    • Fe low
    • Accurate JAK2 mutation
    • Inc basophils
    • Low MCV
    • Tx: Phlebotomy, hydroxyurea
  33. Risks of Pvera
    RCC is assoc with inc hct
  34. Essential thrombocytosis tx
    • If asymptomatic and <60yo and <1.5million plt - no tx
    • >60, thromboisis, or >1.5 million, hydroxyurea
    • Anagrelide for RBC suppression from hydroxyurea
    • ASA for erythromelalgia
  35. Erythromelalgia
    painful red hands in ET
  36. Myelofibrosis tx
    • transplant if young
    • Thalidomide and lenalidomide - TNFi, inc bone marrow production
  37. M3 acute leukemia
    • Translocation btw chrom 15 and 17
    • promyelocytic leukemia
    • risk of DIC
  38. Acute leukemia dx and tx
    • Initial: smear with blasts
    • Auer rods indicate AML
    • Accurate: flow
    • Myeloperoxidas indicates AML
    • Tx: chemo
    • Then if poor prognosis based on cytogenics - bone marrow
    • If good - more chemo
    • Add all-trans-retinoic acid (ATRA) to M3 AML
    • Add intrathecal chemo to ALL
  39. CML presentation
    • pruritus
    • splenomegaly
    • fatigue, night sweats, F
    • routine blood count reveals high WBC
  40. CML dx, tx, risk
    • Initial: Leukocyte alkalne phosphatase (LAP) score is low
    • Accurate: BCR-ABL pcr
    • Tx: TyrKin inh (imatinib/Gleevec, dasatinibm niloptinib. bone marrow for cure
    • 20% conversions per year to acute leukemia
  41. MDS gene, labs
    • 5q deletion - better prognosis
    • Macrocytic anemia, nRBC, blasts, pancytopenia
    • Hypercellular bone marrow
    • Ringed sideroblasts
    • Pelger-Huet cells - bilobed nucleus
  42. MDS tx
    • transfusions
    • erythropoietin
    • lenalidomide if 5q- to dec transfusions
    • Azacitidine dec transfusions but no change in survival
  43. Chronic lymphocytic leukemia sx, dx, risks
    • Asymptomatic, LAD, hepato/splenomegaly, infection
    • WBC>20,000 with >80% lymphocytes
    • Anemia, thrombocytopenia, hypogammaglobulinemia, autoimmune warm IgG, smudge cells
    • Richter phenomenon - conversion to high grade lymphoma, 5%
  44. CLL tx
    • PCP prophylaxis
    • Stage 0/inc WBC or I/LAD: no tx
    • II/hepatosplenomegaly, III/anemia, IV/thrombocytopenia: fludarabine and rituximab
    • Refractory: cyclophosphamide
    • Mild: chlorambucil
    • Severe infection: IVIG
    • Autoimmune thrombocytopenia or hemolysis: prednisone
  45. Hairy cell leukemia: presentation, labs, tx
    • Middle aged men with pancytopenia, splenomegaly, dry tap on bone marrow
    • Bcells with filamentous projections
    • Accurate dx: tartrate resistant acid phosphatase (TRAP) or CD11c
    • Tx: cladribine
  46. NHL dx
    • intial: exicisional bx
    • CBC is mostly normal
    • High LDH = more severe
    • Stage with CT and bone marrow bx
  47. NHL stages and tx
    • I: 1 LN group - local radiation
    • II: 2+ LN - local radiation
    • III: LN on both sides of diaphragm - CHOP and rituximab
    • IV: widespread - "
    • B symptoms - "
  48. CHOP
    • cyclophosphamide
    • adriamycin/doxorubicin
    • vincristine/oncovin
    • prednisone
  49. MALT
    • lymphoma of stomach assoc with Hpylori
    • Clarithromycin and amoxicillin
  50. Hodgkins vs NHL
    • Hodgkins: has Reed-Sternberg
    • more in early stages
    • centers around cervical area
    • NHL: late stages, disseminated
    • Both: same presentations, dx, and staging
  51. Prognosis of lymphomas
    • Hodgkin: Lyphocyte predominant is best and lymphocyte depleted is worst
    • NHL:  Burkitt and immunoblastic have worst prognosis
  52. Hodgkins tx
    • IA: local radiation
    • IIA: local radiation
    • III, IV, B: ABVD
    • Adriamycin/Doxorubicin
    • Bleomycin
    • Vinblastine
    • Dacarbazine
    • Relapses: post rad - chemo. post chemo - higher chemo and bone marrow transplant
  53. Risks of
    radiaton
    chemo
    doxorubicin
    vincristine
    bleomycin
    cisplatin
    • radiaton:solid tumors, premature CAD
    • Chemo: inc risk of acute leukemia, MDS, NHL
    • doxorubicin: cardiomyopathy
    • vincristine: neuropathy
    • bleomycin: lung fibrosis
    • cisplatin: renal and ototoxic
  54. MM dx
    • Initial: xray
    • Serum protein electrophoresis - IgG or IgA M spike
    • HyperCa
    • Bence-Jones proteins
    • Beta2 microglobulin levels indicate severity
    • Smear with rouleaux
    • Inc BUN and Cr
    • >10% plasma cells on bone marrow bx
    • Inc total protein with normal albumin
    • Dec anion gap
    • Normal technetium bone scan
  55. MM tx
    • dexamethasone with lenalidomide and/or bortezomib
    • melphalan is good for old bc less toxic
    • if <70, autologous bone marrow transplant with stem cell support after induction chemo with steroids
  56. MGUS numbers
    • <10% plasma cells on bx
    • 1% transform to MM per year - higher spike increases risk
  57. Waldenstrom Macroglobulinemia - def, presentation, tx
    • IgM overproduction
    • Lethargy, blurry vision, vertigo, engorged eye vessels, mucosal bleeding, raynaud, anemia. no bone lesions
    • tx - chlorambucil, fludarabine and prednisone
  58. Idiopathic thrombocytopenic purpura presentation, dx
    • Thrombocytopenia, normal spleen
    • Exclusion, Antiplatelet Ab, elevated megakaryocytes
  59. ITP tx
    Mild or <30k - steroidsSevere or <10 - IVIG, AntiRhoRecurrent - splenotomy, then romiplostim and eltrombopag, then rituximab, azathioprine, cyclosporine, mycophenolate
  60. vWD presentation, labs, tx
    • minor bleedings, worse with ASA,
    • normal plt, elevated PTT, inc BT, Ristocetin cofactor assay detects vWF dysfx
    • Initial tx: DDAVP (releases stores), VIII replacement, vWF concentrate
  61. Factor XI def
    • inc bleeding after trauma or surgery
    • Normal PT and long PTT which corrects with mixing
    • FFP to stop bleeding
  62. Factor XII deficiency
    • Long PTT but no bleeding
    • no tx
  63. DIC tx
    FFP
  64. HIT
    • 5-10d after start of heparin
    • venous more than arterial clots
    • rarely leads to bleeding
    • confirm with ELISA for platelet factor 4 Ab or seratonin release assay
    • Tx - switch to direct inh - argatroban, lepirudin, bivalidrudin. then warfarin.

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