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

Abstract

Background: 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. Objective: We systematically reviewed diagnostic test accuracy results of bleeding assessment tools (BATs) to screen patients for VWD. Methods: 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 QUADAS-2 and certainty of evidence using the GRADE framework. We pooled estimates of sensitivity and specificity. Results: 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 [CI] 66%-83%) and 54% (29%-77%), respectively. Certainty of evidence varied from moderate to high. Conclusion: 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.

How to investigate mild to moderate bleeding disorders and bleeding disorder of unknown cause

“…Although the ISTH BAT was originally developed and validated for screening of suspected VWD, 20 it has been investigated in other clinical settings including MBD, VWD, BDUC, and IPFD populations. 9,17,[21][22][23] Nevertheless, the bleeding score could not distinguish between patients with BDUC and other MBD diagnoses. 24 In a large study including 359 patients (228 with BDUC, 64%) from the Vienna Bleeding Biobank, the ISTH BAT had a low sensitivity and specificity for the diagnosis of a bleeding disorder.…”

“…The utilization of a standardized bleeding assessment tool (BAT) is essential to evaluate a bleeding tendency. 17 The International Society on Thrombosis and Haemostasis (ISTH) BAT is commonly used. 18 The normal range is ≤3 in men, ≤5 in women and ≤2 in children.…”

“…Although the ISTH BAT was originally developed and validated for screening of suspected VWD, 20 it has been investigated in other clinical settings including MBD, VWD, BDUC, and IPFD populations 9,17,21–23 . Nevertheless, the bleeding score could not distinguish between patients with BDUC and other MBD diagnoses 24 .…”

Reducing use of coagulation tests in a family medicine practice setting: An implementation study

“…The clinical history is an important screening tool for bleeding disorders 9 . To that end, bleeding assessment tools (BATs) such as the Condensed Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM‐1 VWD) Bleeding Questionnaire have been developed and validated in the clinical setting 9–15 . The Condensed MCMDM‐1 VWD BAT provides a structured format to guide clinicians administering a bleeding history.…”

“… 9 To that end, bleeding assessment tools (BATs) such as the Condensed Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM‐1 VWD) Bleeding Questionnaire have been developed and validated in the clinical setting. 9 , 10 , 11 , 12 , 13 , 14 , 15 The Condensed MCMDM‐1 VWD BAT provides a structured format to guide clinicians administering a bleeding history. It has also been shown to have a sensitivity of 100% and negative predictive value of 1 for detecting the most common bleeding disorder, von Willebrand disease (VWD) in adult women, as well as a sensitivity of >99% for other mild bleeding disorders.…”