Bleeding assessment tools in the diagnosis of VWD in adults and children: a systematic review and meta-analysis of test accuracy

• Type of Material: Article
• Authors: Mohamad A. Kalot, SUNY Buffalo; Nedaa Husainat, St Marys Hospital; Sammy Tayiem, University of Kansas; Abdallah El Alayli, University of Kansas; Ahmad B. Dimassi, Lebanese American University; Osama Diab, University of Kansas; Omar Abughanimeh, University of Nebraska Medical Center; Bader Madoukh, SUNY Upstate Medical University; Aref Qureini, University of Missouri Kansas City; Barbara Ameer, Rutgers State University; Jeroen C. J. Di Paola, Washington University St Louis; Jeroen C. J. Eikenboom, Leiden University; Vicky Jacobs-Pratt, Infinity Way; Claire McLintock, Auckland City Hospital; Robert Montgomery, Versiti Blood Center of Wisconsin; James S. O'Donnell, Royal College of Surgeons Ireland; Robert Sidonio Jr, Emory University; Romina Brignardello-Petersen, McMaster University; Veronica Flood, Medical College of Wisconsin; Nathan T. Connell, Brigham & Womens Hospital; Paula D. James, Queens University Belfast; Reem A. Mustafa, University of Kansas
• Language: English
• Date: 2021-12-03
• Publisher: Elsevier
• Publication Version: Final Published Version
• Copyright Statement: © 2021 by The American Society of Hematology.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1182/bloodadvances.2021004368
• Title of Journal or Parent Work: Blood Advances
• Volume: 5
• Issue: 23
• Start Page: 5023
• End Page: 5031
• Grant/Funding Information: None declared
• Keywords: Utility; Hematology; Von-Willebrand factor; Screening tool; Life Sciences & Biomedicine; Validation; Generation; Quality; Guidelines; Science & Technology; Prevalence; Questionnaires; Management
• Research Categories: Health Sciences, Oncology; Health Sciences, Pathology; Health Sciences, Medicine and Surgery

Abstract

Von Willebrand disease (VWD) can be associated with significant morbidity. Patients with VWD can experience bruising, mucocutaneous bleeding, and bleeding after dental and surgical procedures. Early diagnosis and treatment are important to minimize the risk of these complications. Several bleeding assessment tools (BATs) have been used to quantify bleeding symptoms as a screening tool for VWD. We systematically reviewed diagnostic test accuracy results of BATs to screen patients for VWD. We searched Cochrane Central, MEDLINE, and EMBASE for eligible studies, reference lists of relevant reviews, registered trials, and relevant conference proceedings. Two investigators screened and abstracted data. Risk of bias was assessed using the revised tool for the quality assessment of diagnostic accuracy studies and certainty of evidence using the Grading of Recommendations Assessment, Development and Evaluation framework. We pooled estimates of sensitivity and specificity. The review included 7 cohort studies that evaluated the use of BATs to screen adult and pediatric patients for VWD. The pooled estimates for sensitivity and specificity were 75% (95% confidence interval, 66-83) and 54% (29-77), respectively. Certainty of evidence varied from moderate to high. This systematic review provides accuracy estimates for validated BATs as a screening modality for VWD. A BAT is a useful initial screening test to determine who needs specific blood testing. The pretest probability of VWD (often determined by the clinical setting/patient population), along with sensitivity and specificity estimates, will influence patient management.