The 2021 ASH ISTH NHF WFH guidelines recommendation that patients with VWF levels of 30-50 IU/dL and an increased bleeding phenotype be categorized as type 1 VWD rather than Low VWF has proved controversial. However, in support of that decision, recent data have demonstrated that individuals with partial quantitative VWF deficiency exhibit an age-dependent evolving phenotype and confirmed that Low VWF represents a sub-group within heterogeneous type 1 VWD. Nonetheless, type 1 VWD heterogeneity continues to pose significant diagnostic challenges. In this Forum Article, we address outstanding issues critical to preventing the inappropriate overdiagnosis of type 1 VWD, while maximizing access to healthcare and minimizing diagnostic delays. In addition, we propose an algorithm for type 1 VWD diagnosis. This algorithm pays special attention to individuals with plasma VWF levels in the 30-50 IU/dL range who have no or minimal bleeding history and have not yet been exposed to significant hemostatic challenges.
Details
Title
Low von Willebrand factor—unraveling an enigma wrapped in a conundrum
Authors/Creators
James S. O’Donnell
Ross I. Baker - Murdoch University, Centre for Molecular Medicine and Innovative Therapeutics
Ferdows Atiq
Publication Details
Journal of thrombosis and haemostasis, Vol.22(12), pp.3383-3388
Publisher
Elsevier Inc. on behalf of International Society on Thrombosis and Haemostasis.