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The diagnostic workup of von Willebrand disease can be divided into three steps:

  • Identification of patients suspected of having von Willebrand disease, on the basis of data from the clinical history and results of haemostasis screening.
  • Diagnosis of VWD with identification of its type.
  • Characterisation of the subtype.

Clinical history

von Willebrands disease (VWD) should be suspected in any patient with a history of mucocutaneous and postoperative bleeding, especially if the family history suggests an autosomal pattern of inheritance. The most common symptoms associated with VWD are epistaxis, bleedings after dental extractions and menorrhagia.

The bleeding tendency, however, is highly variable and depends on the type and severity of the disease. In many patients with type 1 or type 2 VWD, the bleeding tendency may be absent. In contrast, patients with type 3 VWD have a severe haemorrhagic tendency: mucosal bleedings are very frequent and may be life-threatening. In addition, due to the severe FVIII defect, haematomas and haemarthrosis can occur. Post-operative haemorrhages are also common, especially in patients with very low FVIII levels.

Because of the variability of the clinical history and the relatively low specificity of the bleeding symptoms, the diagnosis of von Willebrand disease also is confirmed on the basis of laboratory tests. 

Flow chart for the diagnosis of a patient with von Willebrand disease (From Federici and Mannucci 1999).


Page updated: 2016-06-17