Work

Assessing the Impact of the 2021 VWD Guidelines on the Diagnosis/Management of Low VWF Patients

Year: 2026

Type: article

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.

Source: Haemophilia

DOI: 10.1111/hae.70275

Authors: Michael Dong, Joanna Larson, Sepideh Saroukhani, Miguel Escobar, Lakshmi Srivaths

Institution: The University of Texas Health Science Center at Houston

Language: English

Topic: Platelet Disorders and Treatments

Subfield: Hematology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $4,760