Rituximab prevents recurrence of thrombotic thrombocytopenic purpura: a case report.
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.
Galbusera
Miriam
M
Mario Negri Institute for Pharmacological Research, Via Gavazzeni, 11, 24125 Bergamo, Italy.
Bresin
Elena
E
Noris
Marina
M
Gastoldi
Sara
S
Belotti
Daniela
D
Capoferri
Cristina
C
Daina
Erica
E
Perseghin
Paolo
P
Scheiflinger
Friedrich
F
Fakhouri
Fadi
F
Grünfeld
Jean-Pierre
JP
Pogliani
Enrico
E
Remuzzi
Giuseppe
G
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
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