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Hemostasis and Thrombosis pp 453–460Cite as

Laboratory Testing for von Willebrand Factor Activity by Glycoprotein Ib Binding Assays (VWF:GPIb)

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Part of the book series: Methods in Molecular Biology ((MIMB,volume 1646))

Abstract

In addition to assessment of von Willebrand factor (VWF) antigen (VWF:Ag), the first-line laboratory investigation of possible von Willebrand disease (VWD) often includes an assay to measure GPIb (glycoprotein Ib) binding activity of VWF. A decreased GPIb binding activity is characteristic for most of the VWD types. For many years, the most frequently used assay for measuring GPIb binding activity was the ristocetin cofactor assay (VWF:RCo), which measures the agglutination of fixed human platelets by VWF in the presence of ristocetin. Because of performance issues, including high assay variability and a lack of VWF sensitivity, this assay is currently being replaced or supplemented by assays based on the binding of VWF to recombinant GPIb. One published method (now abbreviated VWF:GPIbR) uses wild-type GPIb for triggering the binding reaction in the presence of ristocetin. Another more widely used method (now abbreviated VWF:GPIbM) uses gain-of-function GPIb without ristocetin; this permits spontaneous binding of VWF to GPIb and avoids problems associated with the nonphysiological substance ristocetin. The binding of VWF to GPIb can be quantified by using different principles, e.g., ELISA, particle agglutination, or chemiluminescence. The following chapter describes a ristocetin-free method based on particle agglutination in more detail.

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Abbreviations

GPIb:

Glycoprotein Ib

MAB:

Monoclonal antibody

VWD:

von Willebrand disease

VWF:

von Willebrand factor

VWF:CB:

Collagen binding assay

VWF:GPIbM:

von Willebrand factor activity assay based on the spontaneous binding of VWF to a gain-of-function mutant GPIb fragment

VWF:GPIbR:

von Willebrand factor activity assay based on the ristocetin-induced binding of VWF to a wildtype GPIb fragment

VWF:RCo:

Ristocetin cofactor assay

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Author information

Authors and Affiliations

  1. Siemens Healthcare Diagnostics Products GmbH, Assay Development, Emil-von-Behring-Str. 76, 35041, Marburg, Germany

    Jürgen Patzke

  2. Haematology Department, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, NSW Health Pathology, Westmead, NSW, 2145, Australia

    Emmanuel J. Favaloro

Authors
  1. Jürgen Patzke
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  2. Emmanuel J. Favaloro
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Corresponding author

Correspondence to Jürgen Patzke .

Editor information

Editors and Affiliations

  1. Department of Haematology, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, New South Wales, Australia

    Emmanuel J. Favaloro

  2. Section of Clinical Biochemistry, University of Verona, Verona, Italy

    Giuseppe Lippi

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Patzke, J., Favaloro, E.J. (2017). Laboratory Testing for von Willebrand Factor Activity by Glycoprotein Ib Binding Assays (VWF:GPIb). In: Favaloro, E., Lippi, G. (eds) Hemostasis and Thrombosis. Methods in Molecular Biology, vol 1646. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-7196-1_33

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  • DOI: https://doi.org/10.1007/978-1-4939-7196-1_33

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  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4939-7194-7

  • Online ISBN: 978-1-4939-7196-1

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