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Assessing the Impact of the 2021 VWD Guidelines on the Diagnosis/Management of Low VWF Patients
Michael Dong1, Joanna Larson1,2, Sepideh Saroukhani3,4
1Gulf States Hemophilia and Thrombophilia Center Houston, Houston, Texas, USA.
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.
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