Rituximab in the treatment of immune-mediated thrombotic thrombocytopenic purpura

Abstract

PREVIEW

Introduction
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by a deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Lack of ADAMTS13, also known as von Willebrand factor (VWF) cleavage enzyme, results in the formation of microthrombi by VWF and platelets. VWF is a large glycoprotein that plays a key role in platelet adhesion on the vascular wall. Thrombi in the capillaries block blood flow and may result in ischemia in tissue and organs^1-2. The deficiency of ADAMTS13 could be congenital (cTTP), reduced production of ADAMTS13, or reduced ADAMTS13 levels because of antibody formation (immune-mediatedTTP [iTTP])³. The standard treatment of iTTP is plasma exchange (PLEX) and corticosteroid, which have been shown to reduce the mortality rate from 90 to 20%^4-5.