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Tsai H–M – Mutations of the gene are detected in patients with the congenital form of thrombotic thrombocytopenic purpura. In the circulation, proteolysis of von Willebrand factor is critical in regulating vWF–platelet interaction. These advances in knowledge provide the basis for a rational approach to improving the diagnosis and treatment of thrombotic thrombocytopenic purpura.

   

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