Classification of von Willebrand disease in the context of modern contemporary von Willebrand factor testing methodologies

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von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder, potentially affecting up to 1% of the population according to epidemiologic data, although numbers based on presentation to clinics are closer to 0.1%.1 VWD arises from defects and/or deficiency of von Willebrand factor (VWF), and laboratory testing assists clinical exclusion or diagnosis.2-5 Contemporary laboratory assays comprise VWF antigen (VWF:Ag), markers of VWF activity, and factor VIII activity (FVIII:C),2-7 using various methods. There are many VWF activity assays, especially those reflective of glycoprotein Ib (GPIb) binding (including classical ristocetin cofactor [VWF:RCo]), and for which the VWF/VWD Scientific Standardisation Committee (SSC) of the ISTH has recommended revised nomenclature.5, 7 VWF:RCo assays using platelets, ristocetin, and native GPIb remain VWF:RCo, but those using other solid-phase particles and recombinant GPIb are termed VWF:GPIbR.5, 7 Additional gain-of-function VWF assays using recombinant GPIb mutations reflect VWF:GPIb binding assays similar to VWF:RCo and VWF:GPIbR but that do not employ ristocetin are termed VWF:GPIbM.5, 7-9 Another distinct category of VWF activity assay is the collagen binding assay (VWF:CB).5, 10 Assessment of VWF multimers by gel electrophoresis also has a place in VWD diagnosis, although classical methods are nonstandardized, complex, time consuming, and subject to high error rates in diagnostic practice.11, 12

Original languageEnglish
Pages (from-to)952-957
Number of pages6
JournalResearch and practice in thrombosis and haemostasis
Issue number6
Early online date15 Jul 2020
Publication statusPublished - Aug 2020


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