A Case Report of Rituximab Therapy for Recurrent Thrombotic Thrombocytopenia Purpura.
10.5045/kjh.2009.44.3.193
- Author:
Seungbum LEE
1
;
Je Hwan LEE
;
Yunsuk CHOI
;
Ji Hee YU
;
Yoon Hee CHUN
;
Won Jung CHOI
;
Sang Su JUNG
Author Information
1. Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. jhlee3@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Thrombotic thrombocytopenic purpura;
ADAMTS13;
Rituximab
- MeSH:
Adrenal Cortex Hormones;
Anemia, Hemolytic;
Antibodies, Monoclonal, Murine-Derived;
B-Lymphocytes;
Humans;
Neurologic Manifestations;
Plasma Exchange;
Plasmapheresis;
Purpura;
Purpura, Thrombotic Thrombocytopenic;
Thrombocytopenia;
Thymine Nucleotides;
Rituximab
- From:Korean Journal of Hematology
2009;44(3):193-197
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
