Abstract
von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for these VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes several protocols for assessment of VWF activity by means of VWF ristocetin cofactor (VWF:RCo). These assays identify VWF activity by quantitative assessment of VWF protein adhesion to platelets or other particles and subsequent detection of the adhered VWF as facilitated by inclusion of ristocetin. The most commonly performed assays for VWF:RCo comprise platelet agglutination assays, latex agglutination assays, and chemiluminescent assay (CLIA), with three of these described in this chapter.
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Abbreviations
- AVWS:
-
Acquired von Willebrand syndrome
- CLIA:
-
Chemiluminescent assay
- EQA:
-
External quality assessment
- FVIII:
-
Factor VIII
- GP:
-
Glycoprotein
- HMWM:
-
High molecular weight multimers
- RLUs:
-
Relative light units
- VWF:
-
von Willebrand factor
- VWF:Ag:
-
von Willebrand factor antigen
- VWF:CB:
-
VWF collagen binding assay
- VWF:RCo:
-
VWF ristocetin cofactor assay
- VWD:
-
von Willebrand disease
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Acknowledgments
The authors thank current laboratory staff (Jane McDonald, Ella Grezchnik, Monica Ahuja, Shabana Azimulla, and Yifang Zhang) for regular performance of the VWF testing in the Westmead Laboratory. We also thank IL Werfen for previous and ongoing assistance with CLIA-based assay evaluations, as well as for checking the latex- and the CLIA-based VWF:RCo protocols outlined in this paper.
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Mohammed, S., Favaloro, E.J. (2017). Laboratory Testing for von Willebrand Factor Ristocetin Cofactor (VWF:RCo). 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_32
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DOI: https://doi.org/10.1007/978-1-4939-7196-1_32
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