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Low von Willebrand factor-unraveling an enigma wrapped in a conundrum
Journal article   Open access   Peer reviewed

Low von Willebrand factor-unraveling an enigma wrapped in a conundrum

James S. O. ' Donnell, Ross I. Baker and Ferdows Atiq
Journal of thrombosis and haemostasis, Vol.22(12), pp.3383-3388
2024
PMID: 39265913
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Published (Version of Record) Open Access CC BY V4.0

Abstract

aging diagnosis Low VWF type 1 VWD von Willebrand disease
The 2021 ASH ISTH NHF WFH guidelines recommendation that patients with von Willebrand factor (VWF) levels of 30 to 50 IU/dL and an increased bleeding phenotype be categorized as type 1 von Willebrand disease (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 agedependent evolving phenotype and confirmed that Low VWF represents a subgroup 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 to 50 IU/dL range who have no or minimal bleeding history and have not yet been exposed to significant hemostatic challenges.

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