What is von Willebrand disease (vWD)?

Updated: Nov 30, 2019
  • Author: Perumal Thiagarajan, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

Answer

von Willebrand disease (vWD) is the most common inherited bleeding disorder. It is autosomal dominant, and its prevalence is estimated to be as high as one case per 1000 population.

The hallmark of von Willebrand disease is defective platelet adhesion to subendothelial components caused by a deficiency of the plasma protein vWf. This factor is a large, multimeric glycoprotein that is synthesized, processed, and stored in the Weibel-Palade bodies of the endothelial cells, and secreted constitutively and following stimulation.


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