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ADAMTS13, VWF, and a platelet disorder called TTP

Lawrence Brass
From: Science & Medicine: Volume 8 Number 5: Page 278 (October 2002)

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Abstract: Microvascular thrombi occur in patients with thrombotic thrombocytopenic purpura (TTP) and consist predominantly of platelet aggregates. Clotting studies in these patients are typically normal, but unusually large multimers of von Willebrand factor (VWF) are found in blood. Research from several laboratories has recently identified a VWF-cleaving protease, ADAMTS13, that is involved in many cases of TTP. Inhibition or absence of ADAMTS13 prevents the normal processing of VWF after its secretion from endothelial cells, leading to the spontaneous activation of platelets which ultimately results in the microarterial occlusions characteristic of TTP.

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