This guideline is a revision of the separate guidelines for diagnosis and management published in 2004. It is designed to provide a practical approach to the diagnosis and management of von Willebrand disease in the context of managing patients presenting with symptoms of abnormal bleeding. The paper begins with a suggested clinical approach to the patient with bleeding symptoms and incorporates the use of recently developed bleeding scores. It then describes the essential, desirable and optional assessments of von Willebrand factor form and function that are available for laboratory assessment. These are then used to guide suggestions for clinical management. The use of genetic analysis for difficult cases and for genetic counselling are also dealt with.

Declaration of Interests

The UKHCDO paid the expenses incurred during the writing of this guidance. None of the authors had conflicts of interest to declare. All authors have made a declaration of interests to the BSH and Task Force Chairs which may be viewed on request.