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

Christan Urban; Martin Benesch; Petra Sovinz; Wolfgang Schwinger; Herwig Lackner

doi:10.1111/j.1600-0609.2004.00256.x

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

Eur J Haematol. 2004 Jun;72(6):444-7. doi: 10.1111/j.1600-0609.2004.00256.x.

Authors

Christan Urban¹, Martin Benesch, Petra Sovinz, Wolfgang Schwinger, Herwig Lackner

Affiliation

¹ Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Graz, Graz, Austria.

PMID: 15128425
DOI: 10.1111/j.1600-0609.2004.00256.x

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.

Publication types

Case Reports

MeSH terms

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

Substances

Immunoglobulin M
Immunosuppressive Agents