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. Low levels or ineffective von Willebrand factor can cause 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    TOP

vWD is caused by a defect in your genes. This defect differs by the type of vWD:

  • Type 1—results in low levels of von Willebrand factor
  • Type 2—results in von Willebrand factor that does not work well
  • Type 3—results in no von Willebrand factor

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    TOP

Having others in your family with vWD makes your chances of getting this disease higher.

Symptoms    TOP

Many people with the vWD gene don’t 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 events with no obvious cause. This type can also cause bleeding into the joints. This can cause joint swelling and pain.

Diagnosis    TOP

The doctor will ask about your symptoms and health history. Your answers and a physical exam may point to vWD. Blood tests will confirm it and find the type. The tests will also measure the amount of factor in the blood.

Treatment    TOP

Many people with vWD don’t need care. If needed, it depends on the type and seriousness of vWD. In most cases, it’s 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 your 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—iIf antibodies to von Willebrand Factor are developed.

Special steps will also be taken during childbirth for women with vWD. If you’re pregnant, your doctor will watch you. If needed, you will be treated.

Prevention    TOP

There is no way to prevent vWD. If it runs in your family, talk to your doctor about testing. It will help determine the risk of vWD to your child.

RESOURCES:

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

CANADIAN RESOURCES:

Canadian Hemophilia Society
https://www.hemophilia.ca

References:

von Willebrand disease. EBSCO DynaMed website. Available at: http://www.dynamed.... Updated February 1, 2018. Accessed July 12, 2018.
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. Updated January 2017. Accessed July 12, 2018.
Von Willebrand disease. National Heart Lung and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health-topics/von-willebrand-disease. Accessed July 12, 2018.
Von Willebrand disease. National Hemophilia Foundation website. Available at: https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Von-Willebrand-Disease. Accessed July 12, 2018.
Last reviewed June 2018 by EBSCO Medical Review Board Michael J. Fucci, DO, FACC
Last Updated: 7/12/2018

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