Laboratory diagnosis of von Willebrand disease in the age of the new guidelines: considerations based on geography and resources

Emmanuel J. Favaloro, Leonardo Pasalic

Research output: Contribution to journalReview articlepeer-review

4 Citations (Scopus)
28 Downloads (Pure)

Abstract

von Willebrand disease (VWD) is considered the most common bleeding disorder and arises from deficiency and/or defect in the adhesive plasma protein von Willebrand factor (VWF). Diagnosis of VWD requires clinical assessment and is facilitated by laboratory testing. Several guidelines for VWD diagnosis exist, with the latest American Society of Hematology, International Society on Thrombosis and Haemostasis, National Hemophilia Foundation, and World Federation of Hemophilia 2021 guidelines presenting 11 recommendations, some of which have drawn controversy. In the current narrative review, we provide additional context around difficulties in laboratory diagnosis/exclusion/typing of VWD, with a focus on developing countries/resource-poor settings. In particular, there are many variations in assay methodology, and some methods express high assay variability and poor low-level VWF sensitivity that compromises their utility. Although we favor an initial 4-test assay panel, comprising factor (F) VIII coagulant activity, VWF antigen, VWF glycoprotein Ib binding (VWF:GPIbR or VWF:GPIbM favored over VWF Ristocetin cofactor) and VWF collagen binding, we also provide strategies for laboratories only able to incorporate an initial 3-test assay panel, as favored by the latest guidelines, to improve diagnostic accuracy.
Original languageEnglish
Article number102143
Pages (from-to)1-15
Number of pages15
JournalResearch and Practice in Thrombosis and Haemostasis
Volume7
Issue number5
Early online dateJun 2023
DOIs
Publication statusPublished - Jul 2023

Fingerprint

Dive into the research topics of 'Laboratory diagnosis of von Willebrand disease in the age of the new guidelines: considerations based on geography and resources'. Together they form a unique fingerprint.

Cite this