Von Willebrand Factor, ADAMTS13, and the Risk of Mortality

Von Willebrand Factor (VWF) is a multimeric glycoprotein that plays a major role in platelet adhesion and aggregation. Large VWF multimers are highly procoagulant and are cleaved by its metalloprotease ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin motif repeats 13) in smaller, less procoagulant forms. In case of a deficiency of ADAMTS13, large VWF multimers are not cleaved, resulting in more high-molecular VWF multimers in the circulation. This is characteristic for thrombotic thrombocytopenic purpura, in which patients experience microangiopathies, kidney failure, and neurological symptoms because of the formation of microthrombus in the circulation.