Rituximab in the treatment of TTP

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an acute life threatening disorder, for which mortality remains relatively unchanged since the introduction of plasma therapy. Improved understanding of the pathophysiology has identified that the majority of cases result from antibodies directed against ADAMTS 13, which is required to cleave von Willebrand Factor. The use of monoclonal anti-CD 20 therapy removes IgG antibodies, resulting in increased ADAMTS 13 activity, improved time to remission and prevention of recurrent relapses. While further follow-up is required, the side effect profile of anti-CD 20 therapy appears improved compared to conventional immunosuppressive treatments. The use of ADAMTS 13 activity for monitoring can identify patients at risk of a TTP relapse and preemptive therapy with an anti-CD 20 can be considered.