title: Refractory thrombotic thrombocytopenic purpura achieving complete remission with rituximab treatment.
abstract: Some patients with thrombotic thrombocytopenic purpura (TTP) are refractory to standard treatment regimens comprised of plasma exchange (PEX) and steroids. This report describes a 40-year-old woman with refractory TTP who achieved complete remission (CR) in response to rituximab. She was referred to our institution from a rural hospital with purpura of the extremities, severe thrombocytopenia, anemia, and rapidly progressive disturbance of consciousness. TTP was diagnosed based on the clinical symptoms of TTP, low ADAMTS13 activity (<0.5%), and high ADAMTS13 inhibitor (4.4 BU/ml) titers. High-dose prednisolone was immediately administered and PEX was started. This approach was initially effective, but the thrombocytopenia and disturbance of consciousness worsened on the sixth day of treatment. We considered this patient to have refractory TTP and administered weekly rituximab. CR was achieved on day 20, and the disease status of this patient has remained stable over the long term. Our experience with this patient and five others who were similarly treated at our hospital over the past eight years indicates that rituximab is effective for refractory TTP.
authors.affiliation: Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University.
authors.forename: Hiromasa
authors.initials: H
authors.lastname: Iino
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ogawa
authors.forename: Kunio
authors.initials: K
authors.lastname: Yanagisawa
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Shimizu
authors.forename: Takeki
authors.initials: T
authors.lastname: Mitsui
authors.forename: Takuma
authors.initials: T
authors.lastname: Ishizaki
authors.forename: Masaki
authors.initials: M
authors.lastname: Hayakawa
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Nojima
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Handa
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D010951:Plasma Exchange / Q000379:methods; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000188:drug therapy*; D012008:Recurrence; D000069283:Rituximab / Q000008:administration & dosage / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000069283:Rituximab