Von Willebrand disease (VWD) is the most common inherited bleeding disorder.[15]James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):280-300.
https://ashpublications.org/bloodadvances/article/5/1/280/474888/ASH-ISTH-NHF-WFH-2021-guidelines-on-the-diagnosis
http://www.ncbi.nlm.nih.gov/pubmed/33570651?tool=bestpractice.com
According to one systematic review, the reported prevalence of VWD ranges from 108.9 to 2200 per 100,000 people in population-based studies, and from 0.3 to 16.5 per 100,000 people in referral-based studies.[8]Du P, Bergamasco A, Moride Y, et al. Von willebrand disease epidemiology, burden of illness and management: a systematic review. J Blood Med. 2023;14:189-208.
https://www.dovepress.com/von-willebrand-disease-epidemiology-burden-of-illness-and-management-a-peer-reviewed-fulltext-article-JBM
http://www.ncbi.nlm.nih.gov/pubmed/36891166?tool=bestpractice.com
Type 1 VWD is most common (prevalence ranges from 2.7 to 7.2 per 100,000 people based on Italian, UK, and Australian registry data), followed by type 2 (0.8 to 2.5 per 100,000 people) and type 3 (0.1 to 0.3 per 100,000 people).[8]Du P, Bergamasco A, Moride Y, et al. Von willebrand disease epidemiology, burden of illness and management: a systematic review. J Blood Med. 2023;14:189-208.
https://www.dovepress.com/von-willebrand-disease-epidemiology-burden-of-illness-and-management-a-peer-reviewed-fulltext-article-JBM
http://www.ncbi.nlm.nih.gov/pubmed/36891166?tool=bestpractice.com
Type 3 VWD (the most severe type) is more common in countries or regions where consanguineous relationships are accepted.[16]Srivastava A, Rodeghiero F. Epidemiology of von Willebrand disease in developing countries. Sem Thromb Hemost. 2005 Nov;31(5):569-76.
http://www.ncbi.nlm.nih.gov/pubmed/16276466?tool=bestpractice.com
Men and women are affected equally due to the autosomal inheritance pattern of VWD, but adolescent and adult women are more often diagnosed because of the prominent symptom of heavy menstrual bleeding.
Von Willebrand factor (VWF) levels are on average 20% to 30% lower in people with blood group O than in those with a non-O blood group; therefore, people with blood group O are more likely to be diagnosed with VWD.[17]Ward SE, O'Sullivan JM, O'Donnell JS. The relationship between ABO blood group, von Willebrand factor, and primary hemostasis. Blood. 2020 Dec 17;136(25):2864-74.
https://ashpublications.org/blood/article/136/25/2864/461790/The-relationship-between-ABO-blood-group-von
http://www.ncbi.nlm.nih.gov/pubmed/32785650?tool=bestpractice.com
[18]Flood VH, Christopherson PA, Gill JC, et al. Clinical and laboratory variability in a cohort of patients diagnosed with type 1 VWD in the United States. Blood. 2016 May 19;127(20):2481-8.
https://ashpublications.org/blood/article/127/20/2481/35282/Clinical-and-laboratory-variability-in-a-cohort-of
http://www.ncbi.nlm.nih.gov/pubmed/26862110?tool=bestpractice.com
[19]Gill JC, Endres-Brooks J, Bauer PJ, et al. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood. 1987 Jun;69(6):1691-5.
https://www.sciencedirect.com/science/article/pii/S0006497120785484?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/3495304?tool=bestpractice.com