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Laboratory Testing for von Willebrand Disease Using a Composite Rapid 3-Test Chemiluminescence-Based von Willebrand Factor Assay Panel

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Hemostasis and Thrombosis

Part of the book series: Methods in Molecular Biology ((MIMB,volume 2663))

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Abstract

von Willebrand disease (VWD) is the most commonly reported inherited bleeding disorder and may alternatively occur as an acquired von Willebrand syndrome (AVWS). VWD/AVWS develops from defects and/or deficiency in the adhesive plasma protein von Willebrand factor (VWF). VWD/AVWS diagnosis/exclusion remains challenging because of the heterogeneity of VWF defects and the technical limitations of many VWF tests, as well as the VWF test panels (number and type of tests) chosen by many laboratories. Laboratory testing for these disorders utilizes evaluation of VWF level and activity, with activity assessment needing several tests due to the many functions performed by VWF in order to help counteract bleeding. This report explains procedures for evaluating VWF level (antigen; VWF:Ag) and activity by means of a chemiluminescence-based panel. Activity assays comprise collagen binding (VWF:CB) and a ristocetin-based recombinant glycoprotein Ib-binding (VWF:GPIbR) assay that reflects a contemporary alternative to classical ristocetin cofactor (VWF:RCo). This 3-test VWF panel (Ag, CB, GPIbR [RCo]) reflects the only such composite panel available on a single platform and is performed on an AcuStar instrument (Werfen/Instrumentation Laboratory). Certain regional approvals may also allow this 3-test VWF panel to be performed on the BioFlash instrument (Werfen/Instrumentation Laboratory).

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Abbreviations

AVWS:

Acquired von Willebrand syndrome

CLIA:

Chemiluminescent assay

ELISA:

Enzyme-linked immunosorbent assay(s)

EQA:

External quality assessment

FVIII:

Factor VIII

GP:

Glycoprotein

HMWM:

High-molecular-weight multimers

RLUs:

Relative light units

VWD:

von Willebrand disease

VWF:

von Willebrand factor

VWF:Ag:

von Willebrand factor antigen

VWF:CB:

VWF collagen-binding assay

VWF:GPIbB:

VWF GPIb binding

VWF:GPIbR:

VWF glycoprotein I-binding assay using recombinant GPIb

VWF:RCo:

VWF ristocetin cofactor assay

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Acknowledgments

The authors thank the current laboratory and past staff for regular performance of these VWF assays in the ICPMR laboratory. We also thank Werfen/IL for past assistance with CLIA-based assay evaluations performed in our laboratory.

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Authors and Affiliations

  1. Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, NSW, Australia

    Soma Mohammed, Ronny Vong & Leonardo Pasalic

  2. School of Medical Sciences, Faculty of Medicine and Health University of Sydney, Westmead Hospital, Westmead, NSW, Australia

    Emmanuel J. Favaloro

  3. School of Dentistry and Medical Sciences, Faculty of Science and Health, Charles Sturt University, Wagga, Wagga, NSW, Australia

    Emmanuel J. Favaloro

  4. Sydney Centres for Thrombosis and Haemostasis, Westmead, NSW, Australia

    Leonardo Pasalic

  5. Westmead Clinical School, Sydney University, Westmead, NSW, Australia

    Leonardo Pasalic

Authors
  1. Emmanuel J. Favaloro
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  2. Soma Mohammed
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  3. Ronny Vong
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  4. Leonardo Pasalic
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Corresponding author

Correspondence to Emmanuel J. Favaloro .

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Editors and Affiliations

  1. School of Medical Sciences, Faculty of Medicine and Health University of Sydney, Westmead Hospital, Westmead, NSW, Australia

    Emmanuel J. Favaloro

  2. Hemostasis & Thrombosis Center, University of California, Davis, Sacramento, CA, USA

    Robert C. Gosselin

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Favaloro, E.J., Mohammed, S., Vong, R., Pasalic, L. (2023). Laboratory Testing for von Willebrand Disease Using a Composite Rapid 3-Test Chemiluminescence-Based von Willebrand Factor Assay Panel. In: Favaloro, E.J., Gosselin, R.C. (eds) Hemostasis and Thrombosis. Methods in Molecular Biology, vol 2663. Humana, New York, NY. https://doi.org/10.1007/978-1-0716-3175-1_43

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  • DOI: https://doi.org/10.1007/978-1-0716-3175-1_43

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  • Publisher Name: Humana, New York, NY

  • Print ISBN: 978-1-0716-3174-4

  • Online ISBN: 978-1-0716-3175-1

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