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Res Pract Thromb Haemost. 2018 Aug 12;2(4):790-799. doi: 10.1002/rth2.12141. eCollection 2018 Oct.

Evaluation of a semi-automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers.

Author information

1
Department of Coagulation Sheffield Haemophilia and Thrombosis Centre Sheffield UK.
2
Centrum für Blutgerinnungsstörungen und Transfusionsmedizin Bonn Germany.
3
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation Milan Italy.
4
Department of Infection, Immunity and Cardiovascular Disease University of Sheffield Medical School Sheffield UK.

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.

KEYWORDS:

HMWM; Hydragel; VWD; VWF; densitometry; multimer

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