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Semin Thromb Hemost 2022; 48(06): 700-710
DOI: 10.1055/s-0042-1754332
Review Article

Diagnostic Testing for von Willebrand Disease: Trends and Insights from North American Laboratories over the Last Decade

Yonah C. Ziemba
1   Department of Pathology and Laboratory Medicine, Northwell Health, New York, New York
,
Jameel Abdulrehman
2   Department of Medicine, University of Toronto, Toronto, Ontario, Canada
,
Martine J. Hollestelle
3   ECAT Foundation, Voorschoten, the Netherlands
,
Piet Meijer
3   ECAT Foundation, Voorschoten, the Netherlands
,
Elizabeth Plumhoff
4   North American Specialized Coagulation Laboratory Association, Rochester, Minnesota
,
Peihong Hsu
1   Department of Pathology and Laboratory Medicine, Northwell Health, New York, New York
,
Rita Selby
2   Department of Medicine, University of Toronto, Toronto, Ontario, Canada
4   North American Specialized Coagulation Laboratory Association, Rochester, Minnesota
5   Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
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Abstract

Accurate diagnosis of von Willebrand disease (VWD) depends on the quality, precision, and variability of the laboratory assays. The North American Specialized Coagulation Laboratory Association (NASCOLA) is a provider of external quality assessment (EQA) for approximately 60 specialized coagulation laboratories in North America. In this report, NASCOLA EQA data from 2010 to 2021 are reviewed for trends in methodology and precision among various assays. In particular, recent ASH ISTH NHF WFH (American Society of Hematology, International Society on Thrombosis and Haemostasis, National Hemophilia Foundation, and World Hemophilia Federation) guidelines for diagnosis of VWD are reviewed in light of EQA data. In contrast to other geographic regions, laboratories in North America predominantly use three-assay screening panels (antigen, platelet-binding activity, and factor VIII [FVIII] activity) rather than four-assay panels (antigen, platelet-binding activity, FVIII activity, and collagen-binding activity). They also use latex immunoassays rather than chemiluminescence immunoassays, and the classic ristocetin cofactor (VWF:RCo) assay and monoclonal antibody (VWF:Ab) assay to assess VWF platelet-binding activity over newer recommended assays (VWF:GPIbM and VWF:GPIbR). Factors that may be influencing these North American practice patterns include lack of Food and Drug Administration approval of the VWF:GPIbM, VWF:GPIbR, collagen binding assays, and chemiluminescence methodologies, and the influence of the 2008 National Heart, Lung, and Blood Institute guidelines on laboratory practice. Lastly, systems-based solutions are urgently needed to improve the overall accuracy of laboratory testing for VWD by minimizing preanalytical variables and adopting assay standardization.

Keywords

coagulation tests - external quality assessment - proficiency testing - ristocetin cofactor - GPIbM - GPIbR - von Willebrand disease - von Willebrand factor


Publication History

Article published online:
19 September 2022

© 2022. Thieme. All rights reserved.

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