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Thromb Haemost 2000; 84(04): 541-547
DOI: 10.1055/s-0037-1614064
Review Article
Schattauer GmbH

Discrimination of von Willebrands Disease (VWD) Subtypes: Direct Comparison of von Willebrand Factor:Collagen Binding Assay (VWF:CBA) with Monoclonal Antibody (MAB) Based VWF-capture Systems

Emmanuel J. Favaloro
1   From the Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Western Sydney Area Health Service, Westmead, NSW, Australia
,
Anthony Henniker
1   From the Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Western Sydney Area Health Service, Westmead, NSW, Australia
,
David Facey
1   From the Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Western Sydney Area Health Service, Westmead, NSW, Australia
,
Mark Hertzberg
1   From the Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Western Sydney Area Health Service, Westmead, NSW, Australia
› Author AffiliationsThe authors would like to thank all the clinicians that send plasma samples to our laboratory for analysis, and Dr. Jerry Koutts for continued support and encouragement. The technical assistance of Rennie Coombs, who performed the Ristocetin Cofactor assays is also acknowledged. Finally, the staff of the Parramatta blood bank is thanked profusely for continued assistance.
Further Information

Publication History

Received 03 January 2000

Accepted after resubmission 08 May 2000

Publication Date:
11 December 2017 (online)

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Summary

Discrimination of von Willebrand’s Disease (VWD) subtypes is important since it influences management. Qualitative [ie Type 2A, 2B, 2M] defects exhibit von Willebrand factor (VWF) discordance and give high VWF:Ag to VWF:’activity’ ratios. Classically, VWF:’activity’ is assessed using the VWF:RCof assay. The VWF:CBA is an ELISAbased VWF-functional adhesive assay which has consistently proved to be superior to VWF:RCof. A commercially available monoclonal antibody (MAB) based ELISA assay system claimed to mimic a VWF:RCof-like activity has also been recently described (‘SE’), as has the production and characterisation of a large number [n = 10] of locally generated anti-VWF MAB. In the current study, we have adapted these MAB to in-house ELISA assays to assess their utility for VWD diagnosis and subtype discrimination, and to compare them with other assay systems. Thus, the VWF:CBA, VWF:RCof by agglutination, the SE assay, and in-house MAB based assays have been directly compared for their ability to discriminate Type 1 [n = 9] from Type 2 VWD samples [phenotypes 2A and 2B; n = 11]. In summary, MAB-based systems can be used to measure VWF and confirm a diagnosis of VWD, as well as exhibiting some VWD-subtype-discriminatory capabilities. However, better evidence of VWF-discordance was usually achieved using the VWF:RCof (agglutination) assay, while the greatest degree of VWFdiscordance was consistently observed using the VWF:CBA assay. In conclusion, the VWF:CBA assay proved to offer the best diagnostic predictive tool for a Type 2 VWD defect, while MAB-based systems appear to be less effective in this regard.

Abbreviations: HMW: High Molecular Weight [VWF], MAB: Monoclonal antibody (/antibodies), PNP: Pooled Normal Plasma, RIPA: Ristocetin induced platelet aggregation, VWD: von Willebrands disease (/disorder), VWF: von Willebrand Factor, VWF:Ag: von Willebrand Factor Antigen (assay), VWF:CBA: Collagen Binding [Activity] Assay for VWF, VWF:RCof: Ristocetin Cofactor Assay for VWF

Keywords

von Willebrand factor - VWF - von Willebrands disease - von Willebrand disorder - VWD - diagnosis - Collagen binding assay - Collagen - VWF:CBA
 

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