Elevated von Willebrand factor levels during heavy menstrual bleeding episodes limit the diagnostic utility for von Willebrand disease

• Type of Material: Article
• Authors: Megan Brown, Emory University; Michael White, Emory University; Rachel Friedberg, Childrens Healthcare Atlanta; Kalinda Woods, Emory University; Krista Childress, Emory University; Mona Kulkarni, Childrens Healthcare Atlanta; Robert F Sidonio, Emory University
• Language: English
• Date: 2021-05-04
• Publisher: WILEY
• Publication Version: Final Published Version
• Copyright Statement: © 2021 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis (ISTH).
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1002/rth2.12513
• Title of Journal or Parent Work: RESEARCH AND PRACTICE IN THROMBOSIS AND HAEMOSTASIS
• Volume: 5
• Issue: 4
• Start Page: e12513
• End Page: e12513
• Grant/Funding Information: This work was supported by the Georgia Clinical and Translational Science Alliance Postdoctoral Research Training Award (TL1TR002382‐01 and UL1TR002378‐01) and the National Hemophilia Foundation Takeda Clinical Fellowship Program. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health, the National Hemophilia Foundation, or Takeda.
• Keywords: hemostatics; adolescent; menorrhagia; Hematology; DISORDERS; WOMEN; Cardiovascular System & Cardiology; Von Willebrand disease; MENORRHAGIA; blood coagulation tests; MANAGEMENT; von Willebrand factor; Peripheral Vascular Disease; PREVALENCE; Science & Technology; female; Life Sciences & Biomedicine
• Research Categories: Health Sciences, Obstetrics and Gynecology; Health Sciences, Medicine and Surgery

Abstract

Background: Heavy menstrual bleeding (HMB) is often the first bleeding symptom for female individuals with inherited bleeding disorders. Guidelines recommend performing the hemostatic evaluation at HMB presentation. Von Willebrand factor (VWF) levels increase with stress, making it unclear if VWF studies during acute bleeding are beneficial in diagnosing von Willebrand disease (VWD). Objectives: To determine the utility of testing for VWD during acute HMB. Patients/Methods: This retrospective cohort study evaluated VWF levels of individuals presenting to the emergency department (ED) with HMB from January 1, 2017, to December 31, 2018, after prospective implementation of a clinical practice guideline recommending hemostatic evaluation in the ED. We compared VWF and factor VIII (FVIII) levels between acute presentation and follow-up visit after bleeding resolution. We compared the diagnostic accuracy of initial and follow-up labs. Results: During the study period, 221 individuals were seen in the ED for acute HMB, and 39 had VWD testing at both time points. Median FVIII and VWF levels were higher during acute bleeding than at follow-up. The difference in VWF levels between visits was negligible when initial FVIII value was normal. Overall incidence of VWD was 7.5%; 69% of those with VWD had low VWF levels during acute HMB. Conclusion: VWD testing during acute HMB detects the majority of individuals with VWD but also leads to elevated levels of VWF, potentially limiting at the accuracy of diagnostic labs during acute bleeding episodes. Delayed testing until resolution of anemia and active bleeding may provide more accurate diagnostic evaluation for VWD.