title: Rituximab prevents recurrence of thrombotic thrombocytopenic purpura: a case report.
abstract: Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of small vessels that is associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13. The presence of anti-ADAMTS13 autoantibodies is considered a factor predisposing to relapses. Despite close monitoring and intensive plasma treatment, in these patients acute episodes are still associated with substantial morbidity and mortality rates, and the optimal therapeutic option should be prevention of relapses. This study was conducted in a patient with recurrent TTP due to high titers of ADAMTS13 inhibitors, who used to have 2 relapses of TTP a year. The study compared the standard treatment plasma exchange with rituximab. Results documented that plasma exchange had only a small transient effect on ADAMTS13 activity and inhibitors; on the contrary, prophylaxis with rituximab was associated with disappearance of anti-ADAMTS13 antibodies, a progressive recovery of protease activity, and it allowed the patient to maintain a disease-free state during a more than 2-year follow-up.
authors.affiliation: Mario Negri Institute for Pharmacological Research, Via Gavazzeni, 11, 24125 Bergamo, Italy.
authors.forename: Miriam
authors.initials: M
authors.lastname: Galbusera
authors.forename: Elena
authors.initials: E
authors.lastname: Bresin
authors.forename: Marina
authors.initials: M
authors.lastname: Noris
authors.forename: Sara
authors.initials: S
authors.lastname: Gastoldi
authors.forename: Daniela
authors.initials: D
authors.lastname: Belotti
authors.forename: Cristina
authors.initials: C
authors.lastname: Capoferri
authors.forename: Erica
authors.initials: E
authors.lastname: Daina
authors.forename: Paolo
authors.initials: P
authors.lastname: Perseghin
authors.forename: Friedrich
authors.initials: F
authors.lastname: Scheiflinger
authors.forename: Fadi
authors.initials: F
authors.lastname: Fakhouri
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Grünfeld
authors.forename: Enrico
authors.initials: E
authors.lastname: Pogliani
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Remuzzi
mesh_terms: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000097:blood / Q000187:drug effects; D006801:Humans; D007118:Immunoassay; D008297:Male; D008666:Metalloendopeptidases / Q000276:immunology; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy* / Q000628:therapy; D000069283:Rituximab; D055502:Secondary Prevention; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D014841:von Willebrand Factor; D000069283:Rituximab; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human