Von Willebrand Disease | the most common inherited bleeding disorder

Von Willebrand disease (VWD) is a hereditary quantitative deficiency or functional abnormality of von Willebrand factor (VWF), which causes platelet dysfunction. Bleeding tendency is usually mild in the most common types, but it can be severe and present with factor VIII deficiency as well as platelet dysfunction in the rarer subtypes. It is diagnosed based on testing von willebrand factor antigen levels which is low in most subtypes, VWF activity which is low in most subtypes and VWF multimer distribution which is abnormal in a small number of the subtypes. 

Treatment depends on the degree of bleeding. Mild mucosal bleeding can often be managed with tranexamic acid alone to help stabilise the clots that are present. Desmopressin might be used in more significant bleeding or perioperatively. Occasionally von willebrand factor concentrates might need to be given.

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(This isn’t individual medical advice, please use your own clinical judgement and local guidelines when caring for your patients)