Occurrence rates of von Willebrand disease among people receiving care in specialized treatment centres in the United States

• Type of Material: Article
• Authors: John Michael Soucie, Centers for Disease Control and Prevention; Connie H. Miller, Centers for Disease Control and Prevention; Vanessa R. Byams, Centers for Disease Control and Prevention; Amanda B. Payne, Centers for Disease Control and Prevention; Karon Abe, Centers for Disease Control and Prevention; Robert Sidonio Jr, Emory University; Peter A. Kouides, Mary M. Gooley Hemophilia Treatment Center
• Language: English
• Date: 2021-03-29
• Publisher: John Wiley and Sons
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2021 John Wiley & Sons Ltd. Published 2021. This article is a U.S. Government work and is in the public domain in the USA.
• Final Published Version (URL): https://doi.org/10.1111%2Fhae.14263
• Title of Journal or Parent Work: Haemophilia
• Volume: 27
• Issue: 3
• Start Page: 445
• End Page: 453
• Keywords: prevalence; von Willebrand disease; incidence
• Research Categories: Health Sciences, General

Abstract

Introduction: In the network of U.S. comprehensive haemophilia treatment centres (HTCs), von Willebrand disease (VWD) is the most common bleeding disorder other than haemophilia. Estimates of the size and characteristics of the VWD population receiving treatment are useful for healthcare planning. Aim: Estimate the prevalence and incidence of VWD among males and females receiving care at U.S. HTCs (HTC-treated prevalence and incidence). Methods: During the period 2012–2019, de-identified surveillance data were collected on all VWD patients who visited an HTC including year of birth, sex, race, Hispanic ethnicity, VWD type, and laboratory findings and used to calculate period HTC-treated prevalence by VWD type and sex. Data from patients born 1995–1999 were used to estimate HTC-treated incidence rates. Results: During the period, 24,238 patients with a diagnosis of VWD attended HTCs; for 23,479 (96.9%), VWD type was reported or could be assigned. Age-adjusted HTC-treated prevalence was 8.6 cases/100,000 (7.2/100,000 for Type 1, 1.2/100,000 for Type 2 and 1.7/million for Type 3) and was twice as high in women as men (4.8 vs. 2.4 cases/100,000) for Type 1 and similar by sex for Type 2 and Type 3. HTC-treated Type 1 incidence increased over the period, averaging nearly threefold higher for women than men (26.2 vs. 9.9/100,000 live births). Sex differences were less for Type 2 (2.2 vs. 1.4 cases/100,000 births) and slight in Type 3. Conclusion: Prevalence and incidence of HTC-treated VWD differ by sex and type and are likely strongly influenced by differences in rates of diagnosis.