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Von Willebrand Disease (VWD)

Von Willebrand disease (VWD) is a bleeding disorder. It affects your blood's ability to clot. If your blood doesn't clot, you can have heavy, hard-to-stop bleeding after an injury.

PubMed Health Glossary
(Source: NIH - National Heart, Lung, and Blood Institute)

von Willebrand Disease

Von Willebrand disease (VWD) is a bleeding disorder. It affects your blood's ability to clot. If your blood doesn't clot, you can have heavy, hard-to-stop bleeding after an injury. The bleeding can damage your internal organs. Rarely, the bleeding may even cause death.

In VWD, you either have low levels of a certain protein in your blood or the protein doesn't work well. The protein is called von Willebrand factor, and it helps your blood clot.

Normally, when one of your blood vessels is injured, you start to bleed. Small blood cell fragments called platelets (PLATE-lets) clump together to plug the hole in the blood vessel and stop the bleeding. Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot.

Von Willebrand factor also carries clotting factor VIII (8), another important protein that helps your blood clot....Read more about Von Willebrand Disease
NIH - National Heart, Lung, and Blood Institute

What works? Research summarized

Evidence reviews

Drugs that prevent oral bleeding in people with haemophilia or Von Willebrand disease undergoing minor oral surgery or dental extractions

We reviewed the evidence about whether antifibrinolytic medicine (drugs that promote blood clotting) such as tranexamic acid or epsilon aminocaproic acid, can prevent oral bleeding in people with haemophilia or Von Willebrand disease undergoing minor oral surgery or dental extractions.

Treatment of Hemophilia A and B and von Willebrand disease: A Systematic Review [Internet]

Thrombotic adverse events to coagulation factor concentrates for treatment of patients with haemophilia and von Willebrand disease: a systematic review of prospective studies

Thrombotic adverse events (AEs) after clotting factor concentrate administration are rare but the actual rate is unknown. A systematic review of prospective studies (1990-2011) reporting safety data of factor concentrates in patients with haemophilia A (HA), haemophilia B (HB) and von Willebrand disease (VWD) was conducted to identify the incidence and type of thrombotic AEs. In 71 studies (45 in HA, 15 HB, 11 VWD) enrolling 5528 patients treated with 27 different concentrates (20 plasma-derived, 7 recombinant), 20 thrombotic AEs (2 HA, 11 HB, 7 VWD) were reported, including two major venous thromboembolic episodes (both in VWD patients on prolonged replacement for surgery). The remaining thrombotic AEs were superficial thrombophlebitis, mostly occurring at infusion sites in surgical patients and/or during concentrate continuous infusion. The overall prevalence was 3.6 per 10(3) patients (3.6 per 10(4) for severe AEs) and 1.13 per 10(5) infusions, with higher figures in VWD than in haemophilia. Thrombotic AEs accounted for 1.9% of non-inhibitor-related AEs. Thrombosis-related complications occurred in 10.8% of patients with central venous access devices (CVADs) reported in six studies, the risk increasing with time of CVAD use. Data from prospective studies over the last 20 years suggest that the risk of thrombotic AEs from factor concentrate administration is small and mainly represented by superficial thrombophlebitis. These findings support the high degree of safety of products currently used for replacement treatment.

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Summaries for consumers

Drugs that prevent oral bleeding in people with haemophilia or Von Willebrand disease undergoing minor oral surgery or dental extractions

We reviewed the evidence about whether antifibrinolytic medicine (drugs that promote blood clotting) such as tranexamic acid or epsilon aminocaproic acid, can prevent oral bleeding in people with haemophilia or Von Willebrand disease undergoing minor oral surgery or dental extractions.

Desmopressin acetate (DDAVP) for preventing and treating acute bleeds during pregnancy in women with congenital bleeding disorders

We reviewed the evidence about the effect and safety of desmopressin acetate in preventing and treating acute bleeding in pregnant woman with bleeding disorders.

Terms to know

Blood
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Hemophilia
Group of hereditary disorders in which affected individuals fail to make enough of certain proteins needed to form blood clots.
Platelets (Thrombocytes)
A tiny piece of cell that is made by breaking off of a large cell in the bone marrow. Platelets are found in the blood and spleen. They help form blood clots to slow or stop bleeding, and to help wounds heal. Also called thrombocyte.

More about Von Willebrand Disease

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Also called: Von Willebrand's disease, Von Willebrand disorder, Von Willebrand's factor deficiency, vWD

Other terms to know: See all 4
Blood, Genetic, Hemophilia

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