What is the mortality and morbidity associated with pediatric von Willebrand disease (VWD)?

Updated: Feb 05, 2019
  • Author: Suchitra S Acharya, MD, MBBS; Chief Editor: Hassan M Yaish, MD  more...
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Answer

The morbidity in individuals with von Willebrand disease varies. Many children with von Willebrand disease are asymptomatic. Some of these children have cutaneous and/or mucous membrane bleeding (eg, easy bruising, epistaxis).

Menorrhagia is a common symptom in females with von Willebrand disease. It occurs in more than 50% of women with von Willebrand disease and may be the only clinical manifestation of the disease.

The rare type 3 von Willebrand disease can manifest with severe bleeding symptoms similar to those of mild to moderate hemophilia A (eg, hemarthrosis, intramuscular bleeding).

A Swedish study, by Holm et al, found that compared with controls, patients with von Willebrand disease had a higher risk of hospitalization associated with cardiovascular disease (CVD; 1.3 fold) but a lower risk of CVD-linked mortality (0.4 fold). According to the investigators, this suggests that von Willebrand factor deficiency protects against arterial thrombosis even in the presence of atherosclerosis. [8]


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