Bowyer, A.E., Goodfellow, K.J., Seidel, H. et al. (5 more authors) (2018) Evaluation of a semi-automated von willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers. Research and Practice in Thrombosis and Haemostasis, 2 (4). pp. 790-799. ISSN 2475-0379
Abstract
Background The phenotypic diagnosis of von Willebrand disease (VWD) is a multistep process with classification dependent on the quantification of von Willebrand factor (VWF) multimeric structure. VWF multimer analysis is a technically challenging, lengthy and non‐standardised assay, usually performed in specialist laboratories. Recently, a new semi‐automated multimer assay, the Hydragel 5 von Willebrand multimers (H5VWM) has become available.
Objectives This study, performed in two European centres, compared existing in‐house multimer assays to the H5VWM in individuals with and without VWD.
Results Overall agreement of 91.1% was observed in 74 individuals with normal VWF levels, 57 patients grouped as type 1 VWD, 33 type 2A, 16 type 2B, 28 type 2M, 11 type 2N. Patients tested following Desmopressin or VWF concentrate, with thrombotic thrombocytopenic purpura and acquired von Willebrand syndrome were also evaluated. Many of the discrepancies between methods were in patients with genetic mutations linked to more than one type of VWD including p.R1374C/H and p.R1315C. Quantifiable multimer results were available within one working day. Densitometry improved the interpretation of the multimers with slight structural variations that were not apparent by visual inspection of the in‐house method.
Conclusions 5VWM was a rapid, sensitive, standardised assay which used existing technology and could be included as an initial screen of VWF multimers in a VWD diagnostic algorithm in conjunction with traditional multimer analysis.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2018 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals, Inc on behalf of International Society on Thrombosis and Haemostasis. This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. (http://creativecommons.org/licenses/by-nc-nd/4.0/) |
Keywords: | densitometry; HMWM; Hydragel; multimer; VWD; VWF |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Sheffield Teaching Hospitals |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 25 Jun 2018 11:33 |
Last Modified: | 11 Oct 2018 13:18 |
Published Version: | https://doi.org/10.1002/rth2.12141 |
Status: | Published |
Publisher: | Wiley |
Refereed: | Yes |
Identification Number: | 10.1002/rth2.12141 |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:132460 |
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