Aplastic Anemia. Andrew J Avery A.M. Report 04/30/10. Introduction. Aplastic anemia is a syndrome of bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia
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Several retrospective studies suggest that the incidence is 0.6-6.1 cases per million population in the United States
Incidence is much more common in Asia: 4 cases per million in Bangkok, and as high as 14 cases per million in Japan (likely 2/2 environmental factors, as an increased frequency is not seen in persons of Asian ancestry living in the US)
Occurs in all age groups: small peak in childhood 2/2 inherited marrow-failure syndromes; 2nd peak in people aged 20-25 years, and a subsequent peak is observed in people older than 60 years (this 3rd peak may be related to inclusion of MDSs, which are unrelated to aplastic anemia)
Severe aplastic anemia — The criteria for severe aplastic anemia (SAA) are:
A bone marrow biopsy showing <25% of normal cellularity, or
A bone marrow biopsy showing <50% normal cellularity in which fewer than 30% of the cells are hematopoietic and at least two of the following are present: absolute reticulocyte count <40,000/microliter; ANC <500/µL; or plt count <20,000/µL.
Treatment of AA includes withdrawal of potentially offending agents, supportive care (eg, transfusion, antibiotics), and some form of definitive therapy (eg, hematopoietic cell transplantation, immunosuppressive regimens). Blood and platelet transfusions should be used selectively in patients who are candidates for HCT to avoid sensitization
HCT: Allogeneic hematopoietic cell transplantation (HCT) is curative in AA, but is limited by the availability of an HLA-matched sibling as well as by the potentially fatal consequences of graft versus host disease in patients over the age of 40 to 45
Immunosuppressive regimens: Immunosuppressive regimens are not curative, but can be associated with long-term survival