Aplastic Anemia

First Things First (assess & treat for the following)

  • Genetic or acquired marrow disorder with reduction in all marrow cellular elements
    • Technically, a pancytopenia
    • ParvoB19 virus only causes erythrogenic aplasia
  • Marked reduction in CD34 cells responsible for committed progeny and stem cells
  • Qualitative and quantitative dysfunction of the hematopoietic progenitors unresponsive to hematopoietic growth factors
  • Fatty infiltration in marrow
  • Not a disease of stromal elements of marrow
  • Acquired cases are felt to be autoimmune from T-cell destruction of all primitive cell lines, much like other autoimmune disorders.
  • There is a close relationship of aplastic anemia with AML and myelodysplasias.
  • Common etiologies include:
    • Toxicity from medications (chloramphenicol, antineoplastic drugs)
    • Chemicals (benzene, organophosphates, chlorinated hydrocarbons)
    • Viral infections (EBV in 4-6 wks; HIV, HHV-6 and their related drugs, parvoB19 virus)
    • Pregnancy, post-liver transplant, GVHD post-transfusion
    • Radiation-induced
    • Congenital disorders are usually associated with childhood syndromes such as Fanconi’s anemia, Pearson’s syndrome, Diamond-Blackfan syndrome, familial aplastic anemia.
    • Acquired disorders predominate over congenital causes.
  • Eosinophilic fasciitis: autoimmune, connective tissue disorder with aplastic anemia and painful swelling, induration of skin/subcutaneous fascia
    • Minimal response to therapy, antibody-mediated, rare disorder

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Last updated: May 10, 2010