PMID: 9555529Apr 29, 1998Paper

Biology and management of acquired severe aplastic anemia

Current Opinion in Oncology
R A Brodsky

Abstract

Severe aplastic anemia (SAA) is a life-threatening bone marrow failure disorder characterized by pancytopenia and a hypocellular marrow. Drugs, chemical exposure, radiation, and viruses are implicated as etiologic agents, although the majority of community-acquired SAA is idiopathic. Regardless of the inciting event, most cases of SAA result from immune-mediated destruction of bone marrow progenitor cells, which spares pluripotent hematopoietic stem cells. SAA is treated by either allogeneic bone marrow transplantation (BMT) or immunosuppressive therapy. BMT restores normal hematopoiesis and cures the disease in 60% to 80% cases, with the major causes of failure being graft rejection and graft-versus-host disease. Most patients treated with immunosuppressive therapy recover hematopoiesis sufficiently to not require transfusions and are free of infection, although in many, recovery is incomplete. Moreover, up to 50% of SAA patients successfully treated with immunosuppressive therapy relapse or develop a secondary clonal disorder, such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome, or leukemia. High-dose cyclophosphamide without BMT is capable of restoring normal hematopoiesis with little or no risk of relapse ...Continue Reading

Citations

Dec 11, 2002·British Journal of Haematology·Eliane GluckmanUNKNOWN European Group for Blood and Marrow Transplantation Working Party for Severe Aplastic Anemia
Nov 5, 2003·Journal of Hematotherapy & Stem Cell Research·Jiayu ChenDemao Yang
Apr 5, 2005·Immunopharmacology and Immunotoxicology·Marize Campos ValadaresMary L S Queiroz
Mar 7, 2012·Biologicals : Journal of the International Association of Biological Standardization·Sug-Kyun ShinTae-Hwe Heo
Jun 12, 2002·The American Journal of Pathology·Olena JacenkoZhuliang Tao
Jun 18, 1999·Annals of the New York Academy of Sciences·B G ExnerS T Ildstad
Dec 1, 2008·Expert Review of Hematology·Pascal SèveMarc Michel

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