title: [Successful treatment with rituximab in a patient with refractory thrombotic thrombocytopenic purpura refractory to plasma exchange].
abstract: We report a patient with refractory idiopathic thrombotic thrombocytopenic purpura (TTP) who was successfully treated with rituximab. A 50-year-old woman was referred to our hospital with progressive psychoneurotic symptoms, hemolytic anemia and thrombocytopenia. The diagnosis of TTP was confirmed by the absence of ADAMTS13 activity with the presence of circulating ADAMTS13 inhibitor. High-dose steroid therapy and plasma exchange were performed. Despite 21 sessions of plasma exchange, however, there was no remarkable improvement. We then administered rituximab. Fifteen days after the first infusion of rituximab, she achieved complete remission and ADAMTS13 activity increased up to 14%. The patient has remained in remission for more than 9 months.
authors.affiliation: Division of Hematology, Institute of Clinical Medicine, University of Tsukuba.
authors.forename: Mayuko
authors.initials: M
authors.lastname: Koshino
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kudou
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Okoshi
authors.forename: Naoshi
authors.initials: N
authors.lastname: Obara
authors.forename: Seiichi
authors.initials: S
authors.lastname: Simizu
authors.forename: Harumi Y
authors.initials: HY
authors.lastname: Mukai
authors.forename: Kazumi
authors.initials: K
authors.lastname: Suzukawa
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Hasegawa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kojima
authors.forename: Toshiro
authors.initials: T
authors.lastname: Nagasawa
authors.forename: Shigeru
authors.initials: S
authors.lastname: Chiba
mesh_terms: D051722:ADAM Proteins / Q000037:antagonists & inhibitors / Q000097:blood; D000071120:ADAMTS13 Protein; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D015415:Biomarkers / Q000097:blood; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000628:therapy*; D012074:Remission Induction; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D015415:Biomarkers; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human