Types of Von Willebrand Disease Correlate with Severity

There are several types of von Willebrand disease which can range from very mild to severe. In some cases, specific genetic alterations in the von Willebrand factor gene have been identified.

TYPE 1 VON WILLEBRAND DISEASE

Type 1 von Willebrand disease is the most common type of von Willebrand disease, in which a person’s von Willebrand factor (and sometimes factor VIII) is normal in shape, but the amount of protein in the blood is lower than normal.

TYPE 2 VON WILLEBRAND DISEASE

The second most common type of von Willebrand disease is caused by changes to the shape of your von Willebrand factor. Type 2 is subdivided into types 2A, B, M, and N.

  • Type 2A: causes a reduction in platelet binding because of a deficiency of specific von Willebrand factor subunits
  • Type 2B: causes an increase in binding of von Willebrand factor to platelets
    • This results in the platelets and von Willebrand factor subunits being removed from the bloodstream too quickly
  • Type 2M: is a rare type of von Willebrand disease, which causes a decrease in binding of von Willebrand factor to platelets
  • Type 2N: causes a large decrease in binding of von Willebrand factor to factor VIII

TYPE 3 VON WILLEBRAND DISEASE

Type 3 is very rare and is the most severe form of von Willebrand disease. In type 3 von Willebrand disease, levels of von Willebrand factor may be almost zero, and patients often have low factor VIII levels.

PSEUDO VON WILLEBRAND DISEASE

Pseudo (or platelet-type) von Willebrand disease is clinically similar to von Willebrand disease type 2B. Pseudo von Willebrand disease is caused by defects in a person’s platelets rather than problems with their von Willebrand factor.

ACQUIRED VON WILLEBRAND DISEASE

Acquired von Willebrand disease is a form of von Willebrand disease which develops later in life and is not inherited from a parent. It is often associated with an underlying, but unrelated, medical condition.

Learn more about symptoms of von Willebrand disease