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Fatal Evans’ syndrome after matched unrelated donor transplantation for hyper‐IgM syndrome
Author(s) -
Urban Christan,
Benesch Martin,
Sovinz Petra,
Schwinger Wolfgang,
Lackner Herwig
Publication year - 2004
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2004.00256.x
Subject(s) - medicine , evans syndrome , cyclophosphamide , immunoadsorption , transplantation , rituximab , gastroenterology , hemolytic anemia , autoimmune hemolytic anemia , vincristine , cd20 , graft versus host disease , immunology , surgery , antibody , anemia , chemotherapy
A 3½‐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.