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von Willebrand Disease

Definition

von Willebrand disease (vWD) is a genetic blood disorder. It decreases the amount or the effectiveness of von Willebrand factor. This factor is a sticky protein. It helps to make a plug at the site of an injury to stop bleeding. vWD can lead to bleeding problems.
There are 3 major types of vWD:
  • Type 1—most mild and most common form
  • Type 2—less common than type 1
  • Type 3—most serious form and very rare
Genetic Material
Chromosome DNA
Copyright © Nucleus Medical Media, Inc.

Causes

vWD is caused by a gene defect. This defect is most often passed down from the parent.
A child can develop type 1 or type 2 vWD if just one parent has the defect. For type 3, both parents must have the defective gene. Some people may have the gene for vWD without having problems. They can still pass this gene to their children.
vWD can be caused by other health conditions or medicines. This is called acquired von Willebrand syndrome.

Risk Factors

The risk of vWD is higher in people with family members who have it. .

Symptoms

Many people with the vWD gene do not have problems. It may be noticed after an injury or a procedure.
Symptoms usually start in childhood. They tend to waver throughout life. The seriousness will differ from person to person. They may involve:
  • Easy bruising
  • Frequent nosebleeds
  • Bleeding from the gums and minor cuts
  • Heavy bleeding during menstrual periods
  • Blood in the urine
  • Bleeding after childbirth or other trauma
Type 3 vWD can also result in serious bleeding with no obvious cause. This type can also cause bleeding into the joints. This can cause joint swelling and pain.

Diagnosis

The doctor will ask about symptoms and health history. Symptoms and blood tests will confirm it and find the type.

Treatment

Many people with vWD do not need treatment for it. If needed, it depends on the type and seriousness of vWD. Most times, treatment is needed while having a medical or dental procedure.
This may involve:
  • Desmopressin nasal spray or injection—Raises the level of vWF in the blood. It may be used to control bleeding in mild cases of type 1.
  • IV infusions of von Willebrand factors—To control bleeding.
  • Birth control pills—To control heavy menstrual periods in women with type 1 vWD.
  • Antifibrinolytic medicine—For bleeding in the nose or mouth. It keeps a clot from being dissolved before the bleeding has stopped.
  • Recombinant Factor VIIIa—If antibodies to von Willebrand Factor are developed.
Special steps are taken during childbirth for women with vWD.

Prevention

vWD is a genetic disease and cannot be prevented.

RESOURCES

National Heart, Lung, and Blood Institute
https://www.nhlbi.nih.gov
National Hemophilia Foundation
https://www.hemophilia.org

CANADIAN RESOURCES

Health Canada
https://www.canada.ca
Canadian Hemophilia Society
https://www.hemophilia.ca

References

Sharma R, Flood VH. Advances in the diagnosis and treatment of Von Willebrand disease. Blood. 2017;130(22):2386-2391.
von Willebrand disease. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/von-willebrand-disease . Accessed July 20, 2021.
Von Willebrand disease. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/von-willebrand-disease. Accessed July 20, 2021.
Von Willebrand disease. National Heart Lung and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health-topics/bleeding-disorders . Accessed July 20, 2021.
Von Willebrand disease. National Hemophilia Foundation website. Available at: https://www.hemophilia.org/bleeding-disorders-a-z/overview/women-and-bleeding-disorders . Accessed July 20, 2021.

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