Developments in the use of plasma exchange and adjunctive therapies to treat immune-mediated thrombotic thrombocytopenic purpura.

Developments in the use of plasma exchange and adjunctive therapies to treat immune-mediated thrombotic thrombocytopenic purpura. Expert Rev Hematol. 2019 May 16;: Authors: Picod A, Coppo P Abstract INTRODUCTION: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a life-threatening disease characterized by a severe functional deficit in the von-Willebrand cleaving protease ADAMTS13, due to autoantibody production. The once-dismal prognosis of the disease has been changed by the discovery of the dramatic efficiency of therapeutic plasma exchange (TPE). Areas covered: This review focuses on the history and recent developments in the use of TPE for iTTP with a special emphasis on the consequences for TPE practice of the recent introduction of new highly effective immunosuppressive strategies and anti-von Willebrand factor therapies. Expert opinion: Although TPE still represents the cornerstone, emergency treatment of iTTP, their duration, and associated cost and complications could be dramatically reduced in the future by the systematic addition of early immunosuppression using corticosteroids and rituximab as well as an anti-von Willebrand factor therapy with caplacizumab. PMID: 31092093 [PubMed - as supplied by publisher]
Source: Expert Review of Hematology - Category: Hematology Tags: Expert Rev Hematol Source Type: research