Fatal Evans' syndrome after matched unrelated donor transplantation for hyper-IgM syndrome.

Abstract

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.

• Authors: Christan Urban, Martin Benesch, Petra Sovinz, Wolfgang Schwinger, Herwig Lackner
• Journal: European journal of haematology (Eur J Haematol) Vol. 72 Issue 6 Pg. 444-7 (Jun 2004) ISSN: 0902-4441 [Print] England
• PMID: 15128425 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin M
• Immunosuppressive Agents

Topics

• Anemia, Hemolytic, Autoimmune (drug therapy, etiology)
• Blood Donors
• Fatal Outcome
• Graft vs Host Disease (etiology)
• Hematopoietic Stem Cell Transplantation (adverse effects)
• Humans
• Immunoglobulin M
• Immunologic Deficiency Syndromes (complications, therapy)
• Immunosuppressive Agents (therapeutic use)
• Infant
• Male
• Skin Diseases (etiology, immunology)
• Syndrome
• Thrombocytopenia (drug therapy, etiology, immunology)
• Treatment Failure