Abstract

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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 organs1-2. 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.

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Authors

Hasan Tahsin Özpolat - Bloodworks Northwest Research Institute, Seattle, WA, United States of America

Moritz Stolla - Bloodworks Northwest Research Institute, Seattle, WA, United States of America; Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, United States of America; Department of Medicine, Division of Hematology, University of Washington School of Medicine, Seattle, WA, United States of America

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