title: Successful treatment of life-threatening Evans syndrome due to antiphospholipid antibody syndrome by rituximab-based regimen: a case with long-term follow-up.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Haematology and Oncology, Albert-Ludwigs University Freiburg, Hugstetter Strasse 55, Freiburg, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Rückert
authors.forename: H
authors.initials: H
authors.lastname: Glimm
authors.forename: M
authors.initials: M
authors.lastname: Lübbert
authors.forename: C
authors.initials: C
authors.lastname: Grüllich
mesh_terms: D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy* / Q000209:etiology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20 / Q000276:immunology; D016736:Antiphospholipid Syndrome / Q000150:complications*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000069283:Rituximab; D013577:Syndrome; D013921:Thrombocytopenia / Q000188:drug therapy* / Q000209:etiology
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide