Evaluation of a fully automated von Willebrand factor assay panel for the diagnosis of von Willebrand disease

Stufano, Francesca; Baronciani, Luciano; Bucciarelli, Paolo; Boscarino, Marco; Colpani, Paola; Pagliari, M; Peyvandi, Flora

doi:10.1111/hae.13929

Cited by 9 publications

(20 citation statements)

References 34 publications

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“…26 We can only await further clarification of this fascinating story with additional study, in particular with emerging newer contemporary VWF methodologies, including VWF:GPIbR and VWF:GPIbM, as well as novel VWF:CB methods by chemiluminescence procedures. 6,16,18,27 I also look forward to increasing recognition of 2M VWD, and reduction in 2M VWD misdiagnosis. 13,[26][27][28]…”

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confidence: 99%

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

Favaloro

2020

Research and Practice in Thrombosis and Haemostasis

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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][3][4][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 gainof-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 electrophore-This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

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confidence: 99%

“…6,16,18,27 I also look forward to increasing recognition of 2M VWD, and reduction in 2M VWD misdiagnosis. 13,[26][27][28]…”

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confidence: 99%

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

Favaloro

2020

Research and Practice in Thrombosis and Haemostasis

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“…If and when automated/semiautomated platforms become more widely available in the United States, use of these assays may increase. 13,14 We observed a positive bias relative to VWF:RCo for samples categorized as having been run with the HemosIL reagent, presumed to be mostly, if not all VWF:Ab. This affects both the activity measurements and the activity-toantigen ratios and has the potential to affect VWD classification that is based on these ratios.…”

Section: Discussionmentioning

confidence: 80%

“…If and when automated/semiautomated platforms become more widely available in the United States, use of these assays may increase. 13 14…”

Section: Discussionmentioning

confidence: 99%

Analysis of College of American Pathologists von Willebrand Factor Proficiency Testing Program

Salazar

Long

Smock

et al. 2022

Semin Thromb Hemost

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Von Willebrand factor (VWF) level and/or function is altered in von Willebrand disease (VWD), the most common heritable bleeding disorder worldwide. Laboratory assessment of VWF is continually evolving. Historically, the primary method for the assessment of VWF platelet-binding activity was the ristocetin cofactor assay (VWF:RCo). Contemporary alternative measures of VWF platelet-binding activity include VWF:GPIbR (recombinant; using ristocetin), VWF:GPIbM (recombinant; gain-of-function mutant), and monoclonal antibody. Recently, the American Society of Hematology, International Society on Thrombosis and Haemostasis, National Hemophilia Foundation, and World Federation of Hemophilia collaboration issued guidelines recommending the use of newer assays of VWF platelet-binding activity (VWF: GPIbM, VWF: GPIbR) over VWF:RCo, given known limitations of the VWF:RCo assay. Despite this recommendation, the newer VWF:GPIbM and VWF:GPIbR assays are not United States Food and Drug Administration cleared, limiting their availability in the United States. We sought to assess assay utilization trends, agreement of VWF testing methods, and imprecision of VWF testing (based on assigned sample type) from the College of American Pathologists Proficiency Testing Surveys. The analysis confirms that, while VWF antigen testing has low imprecision, the various VWF activity assays have significant interassay variability, with VWF:RCo showing greater imprecision than the newer GPIb-binding assays. The overall trends in assay utilization reflect the barriers to complete compliance with modern VWD diagnostic guidelines in North America.

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“…Jakob et al 295 included the VWF:CB in their study of VWF parameters as potential biomarkers for disease activity and coronary artery lesion in patients with Kawasaki disease, and showed a significant reduction in VWF:CB/Ag ratio in these patients. Stufano et al 296 reported their evaluation of the AcuStar VWF assay panel (VWF:Ag, VWF:GPIbR, VWF:CB) for the diagnosis of VWD. They reported similarly with our prior 2016 study, 249 with AcuStar VWF:GPIbR/Ag and VWF:CB/Ag showing overall good concordance with corresponding data obtained at the time of the historical diagnosis with earlier VWF assays.…”

Section: The Current Decade—the 2020smentioning

confidence: 99%

The Role of the von Willebrand Factor Collagen-Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36-Year History

Favaloro

2023

Semin Thromb Hemost

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The von Willebrand factor (VWF) collagen binding (VWF:CB) assay was first reported for use in von Willebrand diagnostics in 1986, by Brown and Bosak. Since then, the VWF:CB has continued to be used to help diagnose von Willebrand disease (VWD) (correctly) and also to help assign the correct subtype, as well as to assist in the monitoring of VWD therapy, especially desmopressin (DDAVP). However, it is important to recognize that the specific value of any VWF:CB is predicated on the use of an optimized VWF:CB, and that not all VWF:CB assays are so optimized. There are some good commercial assays available, but there are also some “not-so-good” commercial assays available, and these may continue to give the VWF:CB “a bad reputation.” In addition to VWD diagnosis and management, the VWF:CB found purpose in a variety of other applications, from assessing ADAMTS13 activity, to investigation into acquired von Willebrand syndrome (especially as associated with use of mechanical circulatory support or cardiac assist devices), to assessment of VWF activity in disease states in where an excess of high-molecular-weight VWF may accumulate, and lead to increased (micro)thrombosis risk (e.g., coronavirus disease 2019, thrombotic thrombocytopenic purpura). The VWF:CB turns 37 in 2023. This review is a celebration of the utility of the VWF:CB over this nearly 40-year history.

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Evaluation of a fully automated von Willebrand factor assay panel for the diagnosis of von Willebrand disease

Cited by 9 publications

References 34 publications

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

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

Analysis of College of American Pathologists von Willebrand Factor Proficiency Testing Program

The Role of the von Willebrand Factor Collagen-Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36-Year History

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