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 the 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. This chapter describes several protocols for assessment of VWF activity by means of VWF collagen binding (VWF:CB). These assays identify VWF activity by quantitative assessment of VWF protein adhesion to collagen or collagen peptides and subsequent immunological detection of the adhered VWF. The most commonly performed assays for VWF:CB comprise enzyme-linked immunosorbent assays (ELISA) and chemiluminescent assay (CLIA), as described in this chapter.
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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
- TMB:
-
Tetramethylbenzidine dihydrochloride
- VWD:
-
von Willebrand disease
- VWF:
-
von Willebrand factor
- VWF:Ag:
-
von Willebrand factor antigen
- VWF:CB:
-
VWF collagen binding assay
- VWF:RCo:
-
VWF ristocetin cofactor assay
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Acknowledgments
The authors thank the current laboratory staff (Jane McDonald, Ella Grezchnik, Monica Ahuja, Shabana Azimulla, and Yifang Zhang) for regular performance of the VWF:CB ELISA in the Westmead Laboratory. We also thank IL Werfen for the previous and ongoing assistance with CLIA-based assay evaluations as well as for checking the CLIA-based VWF:CB protocol outlined in this chapter.
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Favaloro, E.J., Mohammed, S. (2017). Laboratory Testing for von Willebrand Factor Collagen Binding (VWF:CB). 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_31
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DOI: https://doi.org/10.1007/978-1-4939-7196-1_31
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