Types of von Willebrand Disease

There are 3 main types of von Willebrand disease (VWD)1:

  • Type 1
  • Type 2
  • Type 3

Which type you have depends on the amount of von Willebrand factor (VWF) in your blood and how well it works.

Treatment can vary depending on the type of VWD, so determining the correct type is important.

There is no cure for VWD but with the proper self-care and treatment, children and adults who have the disease can lead healthy, active lives.1

Type 1 VWD

  • The mildest and most common form of VWD, characterized by low levels of VWF1
  • About 60% to 80% of people with VWD have this type2

Type 2 VWD

  • Caused by VWF that does not function properly
  • About 15% to 30% of people with the disorder have type 2 VWD2

    There are 4 subtypes of Type 2 VWD3:

    • Type 2A occurs when there is too little properly formed VWF in the plasma
    • Type 2B occurs when VWF binds too tightly to platelets, potentially reducing the number of platelets in the blood (this is called thrombocytopenia)
    • Type 2M occurs when VWF does not bind tightly enough to platelets (the opposite of 2B)
    • Type 2N is marked by VWF that does not bond properly to factor VIII

Type 3 VWD

Type 3 VWD is a nearly complete absence of VWF. Type 3 VWD is the rarest and most serious form of the disease.3

About 5% to 10% of people with the disease have this type of VWD.2

References

  1. What is von Willebrand disease? National Institutes of Health; National Heart, Lung, and Blood Institute website. http://www.nhlbi.nih.gov/health/health-topics/topics/vwd. Accessed October 21, 2014.
  2. von Willebrand disease. National Hemophilia Foundation website. https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Von-Willebrand-Disease. Accessed October 23, 2014.
  3. Scientific overview. In: The Diagnosis, Evaluation and Management of von Willebrand Disease. Washington, DC: National Institutes of Health; National Heart, Lung, and Blood Institute; 2007. NIH Publication No. 08-5832. https://www.nhlbi.nih.gov/health-pro/guidelines/current/von-willebrand-guidelines/full-report/2-scientific-overview.htm. Accessed October 21, 2014.
Coagulation