How many types of hereditary von Willebrand disease (vWD) are there?

By William Aird

The current classification includes types 1 and 3, which are characterized by quantitative deficiencies of von Willebrand
factor (VWF), as well as types 2A, 2B, 2M, and 2N, which are qualitative variants.

Three main types are:

  • Type 1 – partial quantitative deficiency of vWF.
  • Type 2 – qualitative abnormalities of VWF; 4 subtypes:
    • 2A – characterized by reduced or absent high-molecular-weight vWF.
    • 2B – gain of function in VWF that increases its affinity for platelets.
    • 2M – caused by reduced VWF interactions with platelets or collagen.
    • 2N – results from reduced binding of VWF to FVIII.
  • Type 3 – virtual absence of the VWF protein with associated very low FVIII levels.
Journal of Thrombosis and Haemostasis. 2006;4:2103–2114.
From Leebeek and Eikenboom