Therapeutic plasma exchange in thrombotic thrombocytopenic purpura.

Therapeutic plasma exchange in thrombotic thrombocytopenic purpura. Presse Med. 2019 Nov 20;: Authors: Picod A, Provôt F, Coppo P Abstract Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease related to the formation of microvascular thrombosis and subsequent organ failure. The disease is accompanied with microangiopathic haemolytic anaemia, consumptive thrombocytopenia and lies on a severe deficiency in ADAMTS13, the von Willebrand factor-cleaving protease. In the acquired, immune-mediated form, this deficiency is due to the production of autoantibodies directed against the enzyme. Therapeutic plasma exchange has been used empirically for decades and still represents the cornerstone of TTP treatment. However, a better understanding of pathophysiological mechanisms underlying the disease has led these last years to the development of highly effective targeted therapies that might in the future restraint the use of therapeutic plasma exchange. PMID: 31759790 [PubMed - as supplied by publisher]
Source: Presse Medicale - Category: General Medicine Authors: Tags: Presse Med Source Type: research