title: Long-term remission induced by low-dose rituximab for relapsed and refractory thrombotic thrombocytopenic purpura: A report of two cases.
abstract: Thrombotic thrombocytopenic purpura (TTP) is acquired in the majority of cases. Traditional therapy consists of plasma exchange (PEX), as well as the administration of certain immunosuppressive agents including steroids. A standard dose of rituximab (RTX) at 375 mg/m2weekly for 4 consecutive weeks was recently demonstrated to have significant activity in patients with acquired TTP. To date, clinicians have limited experience using low-dose RTX. In the present study, 2 patients were treated with low-dose RTX at 100 mg weekly for 4 consecutive weeks as a salvage therapy following failure to respond to PEX and other immunosuppressive agents. Prior to RTX therapy, the patients had severely deficient ADAMTS13 activity and detectable anti-ADAMTS13 inhibitors. The patients achieved complete remission and presented long-term stabilization during follow-up. Repeated detection during follow-up demonstrated that the patients had 100% ADAMTS13 activity and undetectable anti-ADAMTS13 antibodies. Although further investigation in a prospective clinical trial is required, the use of low-dose RTX seems to be as effective as a standard dose for patients with relapsing or refractory acquired TTP.
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Zhejiang University, Hangzhou, Zhejiang 310003, P.R. China ; Institute of Hematology, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China.
authors.forename: Hong-Yan
authors.initials: HY
authors.lastname: Tong
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Zhejiang University, Hangzhou, Zhejiang 310003, P.R. China ; Institute of Hematology, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China.
authors.forename: L I
authors.initials: LI
authors.lastname: Ye
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Zhejiang University, Hangzhou, Zhejiang 310003, P.R. China ; Institute of Hematology, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China.
authors.forename: Xing-Nong
authors.initials: XN
authors.lastname: Ye
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Zhejiang University, Hangzhou, Zhejiang 310003, P.R. China ; Institute of Hematology, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China.
authors.forename: DE-Min
authors.initials: DM
authors.lastname: Lu
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Zhejiang University, Hangzhou, Zhejiang 310003, P.R. China ; Institute of Hematology, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Li
keywords: ADAMTS13; low dose; rituximab; thrombotic thrombocytopenic purpura