Treatment of severe Evans syndrome with an allogeneic cord blood transplant.
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.
Raetz
E
E
Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, USA.
Beatty
P G
PG
Adams
R H
RH
D000744:Anemia, Hemolytic, Autoimmune / Q000628:therapy*; D001327:Autoimmune Diseases / Q000628:therapy; D001803:Blood Transfusion; D002675:Child, Preschool; D005312:Fetal Blood / Q000166:cytology*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000628:therapy*; D013577:Syndrome; D014184:Transplantation, Homologous