You are working at a local walk in centre on a busy Saturday afternoon. A patient presents with epistaxis. You are having difficulty in controlling the bleeding and consider packing his nose and transferring him to the nearest Emergency Department. The patient tells you that he suffers from von Willebrand disease.

  1. What is von Willebrand disease?
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Von Willebrand disease (vWD) is the most common hereditary coagulation disorder occurring in approximately 1 in 100 of the population.

It arises from a deficiency in Von Willebrand factor (vWF). This causes a reduction in factor VIII levels as vWF binds to factor VIII to protect it from rapid breakdown within the blood. vWF is also required for platelet adhesion and a deficiency of it will also result in abnormal platelet function. It therefore prolongs both the APTT and the bleeding time. The platelet count and thrombin time are unaffected.

  1. What are the most common symptoms of von Willebrand disease?
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Many patients with vWD are asymptomatic and are diagnosed following a coincidental check of the clotting profile. If symptomatic the most common symptoms are easy bruising, epistaxis and menorrhagia. In severe cases more severe bleeding and haemarthroses can occur.

  1. How is von Willebrand disease treated?
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Desmopressin can be used to treat bleeding in mild cases of von Willebrand disease. This works by raising the patient’s own levels of vWF by releasing vWF that is stored in the Weibel-Palade bodies in the endothelial cells. The Weibel-Palade bodies are the storage granules of endothelial cells that form the inner lining of the blood vessels and the heart.

More severe cases are treated with replacement therapy using cryoprecipitate infusions or Factor VIII concentrate. Replacement therapy is advised for patients with severe von Willebrand disease that are having moderate or major surgical procedures performed.

Header image used on licence from Shutterstock.

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