Coagulation Disorders - Inherited

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Coagulation Disorders - Inherited

  • Von Willebrands Disease is a platelet disorder.
  • This disorder is characterized by a functional defect in Von Willebrands factor (vWF) itself.
  • This disease often clinically manifests with a concurrent deficiency of factor VIII, but will present with a normal platelet count.
  • As far as genetics and inheritance, both men and women are affected equally.
  • Von Willebrands factor is essential for platelet binding, therefore, a defect in vWF causes impaired platelet adhesion and aggegration.
  • The treatment of Von Willebrands Disease involves the administration cryoprecipitate, as it is rich in vWF.