An Evolving Understanding of Low VWF and Type 1 VWD

Author: Connell, NT

Affiliations: Brigham and Women’s Hospital. Harvard Medical School.

Publication: Blood; 2024; 143. 1324–1326

Abstract: In this issue of Blood1 Atiq et al present data to support the hypothesis that low von Willebrand factor (VWF) is an age-dependent evolution of type 1 von Willebrand disease (VWD) rather than its own discrete clinical entity. VWD is the most common inherited bleeding disorder. Diagnosis and accurate subtyping of VWD is complex because of a variety of factors, including physiological stress at the time blood samples are drawn, changes in the hormonal milieu due to menstruation or pregnancy, and other factors, such as specimen integrity and laboratory quality. VWD is classified as either quantitative (types 1 and 3) or qualitative (type 2) defects, with type 1 VWD representing the most common inherited form. Initial testing should include assessment of VWF antigen levels (VWF:Ag), platelet-dependent VWF activity (eg, VWF glycoprotein IbM), and factor.