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von Willebrand Disease
A bleeding disorder of genetic origin, or sometimes acquired, that affects blood clotting.
Von Willebrand disease (vWD) is a genetic bleeding disorder caused by a deficiency or dysfunction of von Willebrand factor (vWF), a key protein involved in blood clotting. vWF is a multimeric protein that enables platelets to adhere to and aggregate at sites of blood vessel injury. It also binds to factor VIII and forms a crucial bridge between platelet receptors and exposed subendothelial collagen, supporting the formation of an effective clot.
What is von Willebrand Disease (vWD)?
Von Willebrand disease (vWD) is classified into three major types:
- Type 1 involves a partial quantitative deficiency of functionally normal von Willebrand factor (vWF), whereas Type 3 is characterized by a complete absence of vWF.
- Type 2 vWD results from qualitative defects in vWF, leading to a disproportionate reduction in vWF activity compared with vWF antigen levels (vWF:Ag). This type is further subdivided into 2A, 2B, 2M, and 2N, based on the specific functional abnormalities of the protein.
- A separate category, acquired von Willebrand syndrome, is not hereditary. It typically develops in adults in association with autoimmune diseases, certain cardiac conditions, some cancers, or other underlying medical disorders.
People with von Willebrand disease (vWD) often experience frequent nosebleeds, easy bruising, and prolonged bleeding during or after invasive procedures such as dental extractions or surgery. Women may have heavy and extended menstrual bleeding, as well as excessive bleeding following childbirth.
Selecting the appropriate treatment depends on the specific vWD subtype and the severity of the condition.
von Willebrand Disease cannot be cured, but effective treatments and self‑care help most people lead active lives. Accurate subtype diagnosis is essential for guiding optimal management.
About 1%
of the population is affected by vWD, and it occurs equally in men and women
1 in 10,000
individuals with vWD show symptoms
16 years
is the average time from first bleeding symptoms to diagnosis
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b) Schneppenheim R, Plendl H, Budde U. Luminography--an alternative assay for detection of von Willebrand factor multimers. Thromb Haemost. 1988 Oct 31;60(2):133-6. PMID: 3064356.
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f) Pikta M, Vasse M, Smock KJ, Moser KA, van DM, Lejniece S, Szanto T, Bautista H, Nouadje G, Banys V. Establishing reference intervals for von Willebrand factor multimers. J Med Biochem. 2022 Feb 2;41(1):115-121. doi: 10.5937/jomb0-31941. PMID: 35431650; PMCID: PMC8970582.
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