Update on ADAMTS13 and VWF in cardiovascular and hematological disorders

Endothelial cells (EC) respond to injury by releasing numerous factors, including von Willebrand factor (VWF). High circulating levels of unusually large VWF multimers (UL-VWFM) have strong procoagulant activity and facilitate platelet adhesion and aggregation by interacting with platelets after an acute event superimposed on peripheral arterial disease and coronary artery disease. ADAMTS13-a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 regulates a key physiological process of coagulation in the circulation by cleaving VWF multimers into small, inactive fragments. Low levels of ADAMTS13 in the blood may play a role in cardiovascular and hematological disorders, and clarifying its role may help improve disease management. The genetic, pharmacological, physiological, and pathological aspects related to ADAMTS13/VWF have been extensively investigated. Here, we provide an update on recent findings of the relationship between ADAMTS13 and hematological/cardiovascular disorders, including thrombotic thrombocytopenic purpura, arterial thrombosis, thrombotic microangiopathy, myocardial infarction, ischemic stroke, heart failure, and hypertension. (C) 2016 Elsevier B.V. All rights reserved.