History and exam
Key diagnostic factors
common
presence of risk factors
Key risk factors include positive family history and consanguineous relationships.
bleeding from minor wounds
Excessive or prolonged bleeding can be an indicator of bleeding disorders, including VWD.
postoperative bleeding
Excessive bleeding and/or having to return to hospital to control re-bleeding may indicate a bleeding disorder. This tends to be more frequent with procedures involving mucous membranes, such as tonsillectomy and dental extractions.
family history of bleeding
A strong indicator for some types of VWD. Usually follows an autosomal-dominant pattern, except for types 2N and 3 disease, which are recessive in inheritance.
easy and excessive bruising
Notably large bruises or bruising on the trunk or other areas not normally exposed to injury can indicate bleeding disorders.
Other diagnostic factors
common
gastrointestinal bleeding
More common in patients with type 2 or 3 disease. Patients should be evaluated for an anatomical cause. Gastrointestinal bleeding is also associated with angiodysplasia, particularly in the context of aortic stenosis and acquired von Willebrand syndrome.
epistaxis
More suggestive if prolonged (>30 minutes) or requires medical intervention to control bleeding.
uncommon
blood transfusions
A history of requiring transfusion repeatedly postoperatively or for significant anaemia can be an indirect indicator of bleeding disorders.
haemarthrosis
Rare except with factor VIII levels <5% of normal.
central nervous system bleeding
Rare, except in patients with severe disease.
haematuria
VWD may exacerbate risk of bleeding from underlying lesions. Patients should be evaluated for an anatomical cause.
Risk factors
strong
positive family history
Heterozygotes for types 2N and 3 are usually asymptomatic.
consanguineous relationships
Severe (type 3) VWD is more common in countries or regions where consanguineous relationships are accepted.[6]
weak
lymphoproliferative disorders
Although rare, acquired von Willebrand syndrome is often associated with lymphoproliferative disorders such as monoclonal gammopathy of undetermined significance, multiple myeloma, or Waldenstrom's macroglobulinaemia.[9]
aortic stenosis
Acquired von Willebrand syndrome has been found to be associated with aortic stenosis.
myeloproliferative disorders
Acquired von Willebrand syndrome has been found to be associated with myeloproliferative disorders.
hypothyroidism
Infrequently, acquired von Willebrand syndrome has been found to be associated with hypothyroidism, as von Willebrand factor synthesis is dependent on thyroid hormone.
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