Von Willebrand disease (vWD) is the commonest inherited bleeding diathesis but may be acquired.
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Clinical presentation
Bruising and mucosal bleeding are typical presentations, but there is a spectrum of severity. The more residual von Willebrand factor (vWF) a patient has, the less severe the bleeding.
Pathology
There are multiple different forms of the disease, but the bleeding is most often due to an autosomal dominant disorder in factor VIII, von Willebrand factor, or both. Lack of the factor leads to decreased platelet activation. Acquired forms of the disease are due to autoantibodies against von Willebrand factor.
Radiographic features
Von Willebrand disease does not have a specific appearance on imaging studies. It can be considered in a setting of unexplained hemorrhage.
One should also take appropriate precaution before considering interventions on patients with this disorder.
History and etymology
It is named after Erik Adolf von Willebrand (1870-1949), Finnish physician and medical professor, who first described this condition in 1926 after studying a family on the Aland islands affected by a bleeding disorder 3. Interestingly he also published on the medicinal benefits of the sauna.
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