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  1. Describe the pathophysiology of aplastic anemia
    • Result of decreased hematopoietic stem cells (decreased ability for self renewal, increased cellular destruction)
    • Decreased signal for cellular proliferation and differentiation
    • No definitive cause is applicable to all causes of aplasia
    • >95% acquired, <5% congenital
  2. Etiology of Acquired Aplastic Anemia
    • Idiopathic or primary
    • 40-70%
    • No definitive cause
    • Secondary
    • Chemical agents (benzene, insecticides, weed killers)
    • Drugs (chemotherapeutical agents, bulsufan, vincristine, colchicine)
    • chloramphenicol
    • phenylbutazone
    • penicillin
    • aspirin
    • ionizing radiation (300-500rads are lethal, lesser doeses are reversible; full recovery in 4-6 weeks)
    • Infections (hepatitis, Epstein-Barr Virus, cytomegalovirus)
    • Micellaneous causes (pregnancy, malnutrition [anorexia], hair dyes [rare])
  3. Clinical features of Acquired Aplastic Anemia
    • Fatigue
    • Dyspnea (abnormal breathing in attempt to get O2)
    • Palpitations (abnormal heartbeat)
    • Infections (no WBC)
    • Bleeding (no platelets) (petechiae [small dots], purprua and ecchymoses [large spots], mucosal bleeding)
  4. What would you find in a Lab examination of Acquired Aplastic Anemia? (peripheral blood values, other tests
    • RBC count: markedly decreased
    • Hgb: <7 g/dL
    • Hct: 20-25%
    • normocytic and normochromic
    • may show A&P
    • reticulocyte count: <1%
    • WBC count: usually <1500/μL
    • decrease in granulocytes and monocytes
    • relative increase in lymphocytes (total amt is less)
    • Platelet count: <20,000-60,000/μL
    • reduced platelets on smear
    • Kidney and liver function tests
    • Blood cultures
    • Bone marrow may be difficult to even aspirate as the majority is replaced with fat
  5. Treatment and prognosis of Acquired Aplastic Anemia
    • Untreated usually results in death
    • Before 1970s treatment: transfusions, steroids, determine etiologic agent
    • More recent: bone marrow transplantation (infused into arm, and stem cells "know where to go"); immunomodulatory therapy using antithymocyte or antilymphocyte globulin
  6. Etiology of Congenital Aplastic Anemia
    • AKA Fanconi's Anemia
    • ~400 clinical cases described (basically nothing)
    • Numerous chr. abnormalities (breakages, defective DNA repair, associated with other hematological abnormalities, acute myelogenous leukemia)
  7. Clinical features of Congenital Aplastic anemia
    • Skeletal defects of thumb
    • Cutaneous hyperpigmentation
    • Renal abnormalities
    • microcephaly
    • mental retardation
    • poor growth
  8. Laboratory examination of Congenital Aplastic anemia
    • Pancytopenia by age 5-10 years
    • Anemia and thrombocytopenia usually precedes leukopenia
    • May have increased Hgb F levels and erythropoieten levels
    • *note- the stress erythropoiesis pattern
    • Poor marrow
  9. Treatment and prognosis of Congenital Aplastic anemia (Fanconi's Anemia)
    • Treatment
    • Some patients respond to cytokine or corticosteroid therapy
    • Allogeneic or cord blood transplantation
    • Prognosis
    • Patients usually die from infections or hemorrhage secondary to the pancytopenia
  10. Information about Pure Red Cell Aplasia
    • Uncommon disorder
    • Over 600 cases identified
    • Selective decrease in RBCs only (erythroid precursor cell)
    • WBCs and platelets are unaffected
    • Acquired and congenital forms
  11. Etiology for Pure Red Cell Aplasia
    • Infections (viruses)
    • drugs
    • malnutrition
    • chronic infections
    • vitamin deficiency in children
    • thymoma or lymphoid neoplasms
    • idiopathic
  12. Laboratory findings for Pure Red Cell Aplasia
    • Severe, chronic, normocytic to slightly macrocytic anemia
    • Decreased reticulocytes (decreased total production of everything)
    • no abnormalities with WBC or platelets
    • No hemolysis or hemmorhage
    • marrow: normal cellularity with noticeable absence of RBC precurors
    • 4:1 M:E
  13. Treatment and prognosis of Pure Red Cell Aplasia
    • Usually recover in a few days to weeks if caused by virus, or with removal of offending drug
    • Surgery may be required to remove offending tumor
  14. all information about Congenital red cell aplasia
    • AKA Diamond-Blackfan anemia
    • May be a molecular defect that prevents RBC precursors from responding to erythropoietin and cytokines
    • Physical abnormalities in head and neck (similar to Fanconi's anemia)
    • Chronic, moderate to severe, normocytic to macrocytic anemia
    • Normal WBCs an platelets
    • Steroid treatment and bone marrow transplantation have been successful in treating the anemia
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2014-03-19 10:50:37
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