Fatal Evans' syndrome after matched unrelated donor transplantation for hyper-IgM syndrome.
A 3 and 1/2-yr-old boy underwent matched unrelated stem cell transplantation (SCT) for hyper-IgM syndrome. He developed acute and chronic skin graft-vs.-host disease (GVHD). Ten months following SCT he presented with severe hemolytic anemia and thrombocytopenia (Evans' syndrome). Treatment included high-dose steroids, intravenous immunoglobulins, cyclosporine, mycophenolate mofetil, chemotherapeutic agents (cyclophosphamide, vincristine, VP-16), immunoadsorption, and anti-CD20 and anti-CD52 monoclonal antibodies without response. The patient died 16 months after SCT.
Urban
Christan
C
Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Graz, Graz, Austria.
Benesch
Martin
M
Sovinz
Petra
P
Schwinger
Wolfgang
W
Lackner
Herwig
H
D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000209:etiology*; D001782:Blood Donors; D017809:Fatal Outcome; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007075:Immunoglobulin M; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000628:therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008297:Male; D012871:Skin Diseases / Q000209:etiology / Q000276:immunology; D013577:Syndrome; D013921:Thrombocytopenia / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D017211:Treatment Failure
D007075:Immunoglobulin M; D007166:Immunosuppressive Agents