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Lupus, Vol. 17, No. 8, 757-760 (2008)
DOI: 10.1177/0961203307087876

case-report

Successful treatment of life-threatening Evans syndrome due to antiphospholipid antibody syndrome by rituximab-based regimen: a case with long-term follow-up

A Rückert

Department of Internal Medicine, Haematology and Oncology, Albert-Ludwigs University, Freiburg, Germany

H Glimm

Department of Internal Medicine, Haematology and Oncology, Albert-Ludwigs University, Freiburg, Germany

M Lübbert

Department of Internal Medicine, Haematology and Oncology, Albert-Ludwigs University, Freiburg, Germany

C Grüllich

Department of Internal Medicine, Haematology and Oncology, Albert-Ludwigs University, Freiburg, Germany, carsten.gruellich{at}uniklinik-freiburg.de

An association of antiphospholipid antibody syndrome with antibodies directed against either phospholipids or plasma proteins strongly suggest that B-cell dysfunction may be involved in its pathogenesis. Antiphospholipid antibody syndrome with autoimmune cytopenias shows a poor response rate to conventional treatment with anticoagulants, glucocorticosteroids, immunosuppressive agents, intravenous immunoglobulin or plasmapheresis. We report a case of life-threatening antiphospholipid antibody syndrome with Evans syndrome receiving successful multimodal treatment including anti-CD20 monoclonal antibody rituximab with long-term follow-up.

Key Words: antiphospholipid antibody syndrome • hemolytic anemia • rituximab • thrombocytopenia


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