Korean J Hematol 2009; 44(3):
Published online September 30, 2009
https://doi.org/10.5045/kjh.2009.44.3.193
© The Korean Society of Hematology
이승범 이제환 최윤숙 유지희 천윤희 최원정 정상수
울산대학교 의과대학 서울아산병원 내과학교실
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/m. 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. (Korean J Hematol 2009;44:193-197.)
Keywords Thrombotic thrombocytopenic purpura, ADAMTS13, Rituximab
Korean J Hematol 2009; 44(3): 193-197
Published online September 30, 2009 https://doi.org/10.5045/kjh.2009.44.3.193
Copyright © The Korean Society of Hematology.
이승범 이제환 최윤숙 유지희 천윤희 최원정 정상수
울산대학교 의과대학 서울아산병원 내과학교실
Seungbum Lee, Je Hwan Lee, Yun suk Choi, Ji Hee Yu, Yoon Hee Chun, Won Jung Choi, Sang Su Jung
Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
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/m. 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. (Korean J Hematol 2009;44:193-197.)
Keywords: Thrombotic thrombocytopenic purpura, ADAMTS13, Rituximab
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