Faculty, Staff and Student Publications

Language

English

Publication Date

1-1-2026

Journal

Haemophilia

DOI

10.1111/hae.70275

PMID

41891800

PMCID

PMC13175425

PubMedCentral® Posted Date

3-27-2026

PubMedCentral® Full Text Version

Post-print

Abstract

Introduction: The 2021 ASH/ISTH/NHF/WFH VWD diagnosis guidelines recommend Type 1 VWD diagnoses for patients with (a) VWF < 0.30 IU/mL or (b) 'Low VWF' 0.30-0.50 IU/mL with the presence of abnormal bleeding. This recommendation recategorizes 'Low VWF' patients with abnormal bleeding into Type 1 VWD.

Aim: To assess the impact of the 2021 VWD guidelines on the diagnosis and management of 'Low VWF' patients.

Methods: A single centre retrospective study was conducted to review all patients presenting for VWD evaluation between January 2000 to September 2024, analyzing for clinical features, ISTH-BAT scores, VWF/FVIII levels, and management of bleeding. 149 patients, totalling 305 clinical encounters, were included in the study.

Results: Seventy five percent of patient encounters had a change in diagnosis from 'Low VWF' to Type 1 VWD after applying the 2021 VWD diagnosis guidelines. The use of VWF therapy (statistically significant, p = 0.04) and antifibrinolytics (not statistically significant, p = 0.52) increased in the post-guidelines period compared to pre-guidelines period. More than half saw increasing VWF levels with age, emphasizing the need to periodically re-evaluate/re-test patients with 'Low VWF'.

Conclusion: Applying the 2021 VWD guidelines leads to improved diagnosis and management of symptomatic patients with 'Low VWF' as they are now classified as Type 1 VWD and receive optimal care with increased use of haemostatic therapy.

Keywords

Humans, Retrospective Studies, Male, Female, von Willebrand Diseases, Middle Aged, Adult, Practice Guidelines as Topic, von Willebrand Factor, Adolescent, Child, Young Adult, Aged, Child, Preschool, Disease Management, low VWF, type 1 VWD, VWD guidelines

Published Open-Access

yes

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