title: [Rituximab provided long-term remission in a patient with severe thrombotic thrombocytopenic purpura refractory to plasma exchange].
abstract: We report a patient with severe thrombotic thrombocytopenic purpura (TTP) refractory to plasmapheresis who was successfully treated with rituximab. A 57-year-old male patient was referred to our department for further differential diagnosis and treatment of anemia and severe thrombocytopenia. Progressive psychoneurotic symptoms, hemolytic anemia, thrombocytopenia, renal function insufficiency and fever led us to the diagnosis of TTP. ADAMTS13 activity was below 3% and an inhibitor for ADAMTS13 was detected. Treatment with plasmapheresis and high-dose steroid was initiated but without clinical benefit. Two weeks following the initiation of plasmapheresis, we decided to treat the patient with 7 cycles of rituximab. No severe rituximab-related adverse effects were observed. After treatment with rituximab, the disease remitted, and the ADAMTS13 activity level increased. The patient has remained in complete remission for more than 1 year. Our data suggest that rituximab may be the optimal immunosuppressive therapy for refractory thrombotic thrombocytopenic purpura caused by an anti-ADAMTS 13 inhibitor.
authors.affiliation: Department of Hematology, Seichokai Fuchu Hospital.
authors.forename: Hyunsoo
authors.initials: H
authors.lastname: Hong
authors.forename: Yasutaka
authors.initials: Y
authors.lastname: Aoyama
authors.forename: Ryousuke
authors.initials: R
authors.lastname: Yamamura
authors.forename: Tadanobu
authors.initials: T
authors.lastname: Ohta
authors.forename: Atsuko
authors.initials: A
authors.lastname: Mugitani
authors.forename: Takahisa
authors.initials: T
authors.lastname: Yamane
authors.forename: Masayuki
authors.initials: M
authors.lastname: Hino
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
mesh_terms: D051722:ADAM Proteins / Q000037:antagonists & inhibitors / Q000172:deficiency; D000071120:ADAMTS13 Protein; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology / Q000628:therapy*; D012074:Remission Induction; D000069283:Rituximab; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human