Summary

• Autosomal inheritance with variable penetrance and phenotypic expression.
• Usually presents with mucocutaneous bleeding.
• Menorrhagia and postpartum haemorrhage common in affected females.
• Joint bleeding rare and seen only in patients with more severe disease.
• Most patients have type 1 von Willebrand disease; more severe symptoms are seen in types 2 and 3.
• Clinical bleeding scores may be helpful in identifying patients with disease.