1. Fatal Evans’ syndrome after matched unrelated donor transplantation for hyper-IgM syndrome.
- Author
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Urban, Christan, Benesch, Martin, Sovinz, Petra, Schwinger, Wolfgang, and Lackner, Herwig
- Subjects
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STEM cell transplantation , *CELL transplantation , *HEMOLYTIC anemia , *ANEMIA , *HEMOLYSIS & hemolysins , *BLOOD diseases - Abstract
Urban C, Benesch M, Sovinz P, Schwinger W, Lackner H. Fatal Evans’ syndrome after matched unrelated donor transplantation for hyper-IgM syndrome. Eur J Haematol 2004: 72: 444–447. © Blackwell Munksgaard 2004. 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. [ABSTRACT FROM AUTHOR]
- Published
- 2004
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