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KMID : 0366220090440030193
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Korean Journal of Hematology
2009 Volume.44 No. 3 p.193 ~ p.197
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A Case Report of Rituximab Therapy for Recurrent Thrombotic Thrombocytopenia Purpura
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Lee Seung-Bum
Lee Je-Hwan
Choi Yun-Suk
Yu Ji-Hee
Chun Yoon-Hee
Choi Won-Jung
Jung Sang-Su
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Abstract
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Thrombotic thrombocytopenic purpura (TTP) is a rare medical condition characterized by thrombocytopenia, microangiopathic hemolytic anemia, neurologic manifestations, and infrequently, renal involvement. In many cases, TTP is associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13, and treatment with plasma exchange is effective in the majority of patients. We report a patient with acute TTP in whom remission was not achieved by initial treatment consisting of plasma exchange and corticosteroids. In view of the severe autoantibody-mediated ADAMTS-13 deficiency, treatment was initiated with rituximab, a chimeric monoclonal antibody directed against the CD 20 antigen present on B lymphocytes. The patient received 4 weekly infusions of rituximab (375 mg/m2). Four weeks after the last infusion of rituximab, a complete clinical and laboratory remission was documented. We conclude that rituximab should be considered in patients with TTP with acquired ADAMTS-13 deficiency, who fail to respond to standard treatment with plasma exchange and corticosteroids. Rituximab may result in a lowered requirement for plasmapheresis and avoid the complications of salvage immunosuppressive therapy.
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KEYWORD
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Thrombotic thrombocytopenic purpura, ADAMTS13, Rituximab
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