Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura

Blood. 2014 Jul 10;124(2):204-10. doi: 10.1182/blood-2014-01-550244. Epub 2014 May 28.

Abstract

In acquired thrombotic thrombocytopenic purpura (TTP), the persistence of severe ADAMTS13 deficiency (<10%) during remission is associated with more relapse. Preemptive (ie, after remission) administration of rituximab in these patients to prevent relapses remains controversial. We performed a cross-sectional analysis of 12-year follow-up data to compare the relapse incidence with or without preemptive rituximab infusion. Among 48 patients who experienced at least one episode of acquired TTP followed by severe ADAMTS13 deficiency during remission, 30 received preemptive rituximab (group 1); the other 18 did not (group 2). After a median of 17 months (interquartile range [IQR], 11-29) following rituximab, the relapse incidence decreased from 0.57 episodes/year (IQR, 0.46-0.7) to 0 episodes/year (IQR, 0-0.81) (P < .01) in group 1. ADAMTS13 activity 3 months after the first rituximab infusion increased to 46% (IQR, 30%-68%). Nine patients required additional courses of rituximab. In 5 patients, ADAMTS13 activity failed to increase durably. Four patients experienced manageable adverse effects. In group 2, the relapse incidence was higher (0.5 relapses/year; IQR, 0.12-0.5; P < .01). Relapse-free survival was longer in group 1 (P = .049). A persistent severe ADAMTS13 deficiency during TTP remission should prompt consideration of preemptive rituximab to prevent relapses.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • ADAM Proteins / blood
  • ADAM Proteins / deficiency
  • ADAM Proteins / immunology
  • ADAMTS13 Protein
  • Adult
  • Antibodies, Monoclonal, Murine-Derived / administration & dosage*
  • Autoantibodies / blood
  • Chemoprevention / methods
  • Cross-Sectional Studies
  • Female
  • Humans
  • Infusions, Intravenous
  • Male
  • Purpura, Thrombotic Thrombocytopenic / blood
  • Purpura, Thrombotic Thrombocytopenic / prevention & control*
  • Remission Induction
  • Retrospective Studies
  • Rituximab
  • Secondary Prevention
  • Treatment Outcome

Substances

  • Antibodies, Monoclonal, Murine-Derived
  • Autoantibodies
  • Rituximab
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human

Supplementary concepts

  • Thrombotic thrombocytopenic purpura, acquired