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What is von Willebrand Disease?

Learn about the most common bleeding disorder

Imbalanced scale showing how a bleeding tendency relates to bleeding disorders.

What is von Willebrand disease?

Von Willebrand disease (VWD) is a blood disorder in which the blood does not clot properly. Blood is made up of several proteins that, in healthy individuals, interact with each other in a common, sequential order after an injury. Von Willebrand factor is one of these proteins. It plays an important role in helping blood to clot by mediating the attachment of platelets to the injured vessel wall.

Affected people either have a low level of von Willebrand factor (VWF) in their blood or the VWF protein is not fully functional. In these cases, the clot can take longer to form or forms incorrectly, and bleeding might take longer to stop. This can lead to heavy, hard-to-stop bleeding. Significant bleeding symptoms due to reduced VWF levels have been reported in approximately 1 in 1000 individuals.1,2



Types

There are three major types of VWD:3

Type 1 

(~75% of VWD cases)

The mildest and most common form of VWD. Affected people have low levels of VWF in their blood with few to no bleeding symptoms.

Type 2 

(~25% of VWD cases)

VWF has a defect that makes it unable to work properly. Patients have mild or moderate bleeding symptoms.

Type 3 

(<0.1% of VWD cases)

The most severe and rarest type of VWD. Affected people have no or very low amounts of VWF in their blood. 

Causes

An illustration of a family tree showing how von Willebrand Disease is inherited from parent to child, with colors indicating the severity.

Most people with von Willebrand Disease have inherited it from a parent.

How do you get von Willebrand Disease?

VWD is the deficiency and/or malfunction of VWF. It is almost always inherited from a parent by a child, which means that most affected people are born with it. People with inherited VWD have a defective gene involved in the production of von Willebrand factor, which leads to a reduction in VWF or a decrease in its functionality.

The much rarer form of VWD is acquired von Willebrand syndrome (AVWS), with only a few hundred reported cases.4 AVWS is caused mainly by malignant leukocyte disorders (e.g., lymphoma/leukemia), heart defects, autoimmune diseases (collagenoses or antibodies against VWF), or certain drugs (e.g., valproic acid (in children), ciprofloxacin, etc.).

Symptoms

Nosebleed

Frequent nosebleeds may be a symptom of VWD.

What are the symptoms associated with von Willebrand disease?5

  • Excessive bleeding from an injury or after surgery or dental work
  • Frequent nosebleeds that do not stop within 10 minutes
  • Heavy or long menstrual bleeding
  • Heavy bleeding during labor and delivery
  • Blood in urine or stool
  • Easy bruising or lumpy bruises 

illustration of the female reproductive system

Heavy or long menstrual bleeding may be caused by von Willebrand disease.

Women and von Willebrand Disease

Menstrual signs and symptoms of VWD might include:5
  • Blood clots greater than 1 inch in diameter in your menstrual flow
  • The need to change your menstrual pad or tampon more than once an hour
  • The need to use double sanitary protection for menstrual flow
  • Symptoms of anemia, including tiredness, fatigue, or shortness of breath

Doctor Icon

When should a doctor be called?

A doctor should be consulted when you experience bleeding that lasts a long time or is difficult to stop. Women with von Willebrand disease should speak to their doctor when considering pregnancy. The doctor can recommend precautions to lower the risk of complications.

Diagnosis

Patient and physician talking about blood health

Speak with your physician if you experience symptoms of a bleeding disorder.

How is von Willebrand disease diagnosed?

Arriving at a diagnosis of von Willebrand disease is a complex process that may require several physician visits and laboratory tests. The first step in diagnosis is identifying a positive bleeding history or a family history of bleeding. 

The next step is based on laboratory tests, where the first investigation would be comprised of the following tests:

  • VWF concentration
  • VWF activity
  • Coagulation factor VIII activity
Further evaluations can include molecular genetic diagnostics. 

Treatment

How is von Willebrand disease treated?

VWD has no cure, but the right treatment can help to stop or prevent bleeding. Treatment for VWD depends on the type and severity of the disease. Patients with VWD are treated only if active bleeding is present or invasive procedures (e.g., surgical procedures or tooth extractions) are scheduled. 

Medical tablet and medical pill

Strategies to prevent or control bleeding in people who have VWD:6

  • Nonreplacement therapy: Increase plasma concentration of VWF through stimulation of endothelial cells with DDAVP (desmopressin).
  • Replacement therapy: Replace VWF by using human plasma-derived concentrates of coagulation factors.

Other strategies:6 

Promote hemostasis and wound healing without altering the plasma concentration of VWF:

  • Antifibrinolytics (e.g., aminocaproic and tranexamic acid) are agents that inhibit fibrinolysis and thus the development of bleeding. They can also play an important role in the treatment of heavy menstrual bleeding.
  • Tranexamic acid has been shown to decrease menstrual blood loss by 50%.7


We stand behind you

We hope you learned something about von Willebrand disease today!

Stay safe and healthy!

The information provided here is a brief, simplified explanation of von Willebrand factor and von Willebrand disease. For more details, please consult scientific textbooks or publications.

1

Nosek-Cenkowanska B, et al. Thromb Heamost. 1991. doi.org/10.1055/s-0038-1647491

2

Sadler JE, et al. Thromb Haemost. 2000. doi.org/10.1055/s-0037-1613992

3

Fogarty H, et al. BJHaem. 2020. doi.org/10.1111/bjh.16681

4

Federici AB, et al. Thromb Haemost. 2000. doi.org/10.1055/s-0037-1614018

7

Weyand A, Flood VH. HOC. 2021. doi.org/10.1016/j.hoc.2021.07.004