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Treatment of severe Evans syndrome with an allogeneic cord blood transplant.
Bone Marrow Transplantation 1997 September
Immunosuppressive therapy is commonly used in the management of autoimmune disorders. As marrow-derived lymphocytes appear to play a key role in these diseases, lymphoid ablation followed by replacement with autologous or allogeneic stem cells may be a therapeutic option. We report a 5-year-old boy with severe Evans syndrome which consists of immune thrombocytopenia and Coombs-positive hemolytic anemia. He was rendered into complete remission with marrow ablation followed by rescue with an HLA-identical sibling cord blood transplant. He unexpectedly died 9 months following transplant from acute hepatic failure of unknown etiology.
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