Causes of von Willebrand disease

What can lead to such a complex bleeding disorder?

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Von Willebrand disease causes & genetics

Our bodies produce a number of different clotting factors that work together in a sequence to produce a blood clot. When the body can’t produce enough of the protein called von Willebrand factor (VWF), or it doesn't work properly, the body can’t control bleeding.1

VWF does two very important things to help the body form blood clots.1

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It acts like glue to help platelets stick together and form a platelet plug at the site of injury.

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It acts like a chaperone for factor VIII: it helps protect, stabilize, and carry factor VIII to the site of injury so it can help make blood clots.

Inherited VWD vs acquired VWD

Von Willebrand disease can be inherited in three different ways: from either the mother or the father or both parents.2

Though rare, it is possible for someone to develop VWD later in life, when a person’s own immune system destroys his or her VWF. This might happen as a result of another disease or use of a medication. Acquired VWD can't be passed on to any children.3

How VWD is inherited4

The gene for VWF is not on the sex gene, so VWD occurs equally often in males and females. It is not always easy to predict who will develop VWD, but some helpful basics are explained below.

  • VWD types 1 and 2 are usually inherited in what is known as a "dominant" pattern. This means that to have these types of VWD, the child needs to inherit one faulty gene only—from one parent. That gene is "dominant"
  • Type 3 VWD is usually inherited in a "recessive" pattern. This means that in order to develop VWD of this type, the child needs to inherit two faulty genes—one from each parent

Learn more about VWD types

Dominant inheritance pattern diagram for VWD Types 1 and 2.

A child will have VWD if he or she inherits the dominant gene from either parent.

Recessive inheritance pattern diagram for VWD Type 3.

A child will have VWD if he or she inherits the recessive gene from both parents.

Key for the inheritance pattern diagrams.

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  1. James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021;5(1):280-300. doi:10.1182/bloodadvances.2020003265
  2. Canadian Hemophilia Society. All about von Willebrand disease…for people with von Willebrand disease and their families. http://www.hemophilia.ca/files/AllAboutVWDRevisedEng.pdf. Accessed February 12, 2023.
  3. Centers for Disease Control and Prevention. What is von Willebrand disease? http://www.cdc.gov/ncbddd/vwd/facts.html. Accessed February 16, 2023.
  4. Hemophilia of Georgia. The inheritance of von Willebrand disease in The Hemophilia, von Willebrand Disease & Platelet Disorders Handbook. https://www.hog.org/handbook. Accessed February 12, 2023.