[Update on thrombotic thrombocytopenic purpura]

Rev Med Interne. 2008 Oct;29(10):794-800. doi: 10.1016/j.revmed.2007.09.034. Epub 2007 Oct 22.
[Article in French]

Abstract

Purpose: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder and early treatment is vital. Here, we review the recent advances in the understanding of the pathophysiology of TTP and its treatment.

Current knowledge and key points: Recent advances have shown that TTP is caused by deficiency of the (ADAMTS-13) metalloprotease that cleaves von Willebrand factor multimers. Acquired TTP is associated to inhibitory antibodies directed against ADAMTS-13. This has led to assess new therapeutic approaches in refractory and relapsing forms of TTP and the use of rituximab has shown very encouraging results.

Future prospects and projects: A better characterization of TTP amongst the other thrombotic microangiopathies has allowed the use of new therapeutic approaches with the use of rituximab. The encouraging results reported with rituximab in some forms of TTP challenge the classic treatment based on plasma exchanges.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Diagnosis, Differential
  • Humans
  • Plasma Exchange
  • Platelet Aggregation Inhibitors / therapeutic use
  • Purpura, Thrombotic Thrombocytopenic / diagnosis*
  • Purpura, Thrombotic Thrombocytopenic / epidemiology
  • Purpura, Thrombotic Thrombocytopenic / physiopathology
  • Purpura, Thrombotic Thrombocytopenic / therapy*

Substances

  • Adrenal Cortex Hormones
  • Platelet Aggregation Inhibitors