title: Long-term response to rituximab in patients with relapsing thrombotic thrombocytopenic purpura.
abstract: Acquired thrombotic thrombocytopenic purpura (TTP) is an uncommon disease in adults, characterized by fever, neurological manifestations, microangiopathic hemolytic anemia, thrombocytopenia, renal dysfunction, and the presence of antibodies against the enzyme ADAMTS13. Treatment with plasmapheresis has increased the survival from 10% to more than 90%. Still, there is a subset of patients with resistant TTP who fail to respond to plasmapheresis or remain dependent on this procedure. There is mounting evidence that rituximab may play an important role in remission induction of resistant/relapsing TTP, but the extent of the remission is unknown. We present here four patients with chronic-relapsing TTP who responded favorably to rituximab. All four patients achieved prolonged remission of 23 to 82 months after the treatment. One patient relapsed 6 years afterthe initial treatment with rituximab and re-entered remission following retreatment.
authors.affiliation: Department of Internal Medicine B, Rabin Medical Center (Beilinson Campus), Petah Tikva, affiliated with Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel.
authors.forename: Gideon Y
authors.initials: GY
authors.lastname: Stein
authors.forename: Dorit
authors.initials: D
authors.lastname: Blickstein
authors.forename: Jerome
authors.initials: J
authors.lastname: Orlin
authors.forename: Galit
authors.initials: G
authors.lastname: Sarig
authors.forename: Aida
authors.initials: A
authors.lastname: Inbal
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D018951:Antigens, CD20; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab; D013997:Time Factors; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D007155:Immunologic Factors; D000069283:Rituximab