title: Rituximab therapy to prevent relapse in chronic relapsing thrombotic thrombocytopenic purpura (TTP) in a child.
abstract: Our patient first developed thrombotic thrombocytopenic purpura (TTP) at age 10 years with an initial platelet count of 10,000/microL. She achieved remission with plasmapheresis (PE), but suffered 2 relapses in the next 2 years, each approximately 1 year from PE, with ADAMTS13 levels of <5%. Early in her third remission, with vincristine (weekly x 4 doses) and prednisone (for 2 weeks) her ADAMTS13 increased to 99% in 24 weeks, but decreased to <4% in the next 38 weeks. After 4 weekly doses of rituximab (375 mg/m(2)), her ADAMTS13 level reached 101% in 9 weeks and has remained consistently above 97% on bimonthly monitoring for more than a year. She remains in continuous clinical and hematologic remission with an ADAMTS13 level of 108% at 60 weeks from rituximab therapy and 124 weeks from her second relapse. This case report suggests that monitoring ADAMTS13 level at regular intervals in recurrent TTP may help us identify patients at risk for further relapse; and such a relapse may be prevented, or at least delayed with timely rituximab therapy, thus reducing morbidity from relapsed TTP and its treatment.
authors.affiliation: Department of Pediatrics, New York Medical College, Valhalla, New York 10595, USA. s jayabose@nymc.edu
authors.forename: Somasundaram
authors.initials: S
authors.lastname: Jayabose
authors.forename: Julie
authors.initials: J
authors.lastname: Dunbar
authors.forename: Theodore S
authors.initials: TS
authors.lastname: Nowicki
authors.forename: Oya
authors.initials: O
authors.lastname: Tugal
authors.forename: Mehmet Fevzi
authors.initials: MF
authors.lastname: Ozkaynak
authors.forename: Claudio
authors.initials: C
authors.lastname: Sandoval
mesh_terms: D051722:ADAM Proteins / Q000378:metabolism; D000071120:ADAMTS13 Protein; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002648:Child; D002908:Chronic Disease; D005260:Female; D006801:Humans; D009364:Neoplasm Recurrence, Local / Q000378:metabolism / Q000473:pathology / Q000517:prevention & control*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D012074:Remission Induction; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human