Abstract Number: PB0922
Meeting: ISTH 2021 Congress
Background: The VWF multimer (VWF:MM) assay is an important tool while evaluating patients with suspected Von Willebrand Diseases (VWD), but results are rarely available in a timely manner. A rapid, automated standardized assay was developed to overcome this important limitation.
Aims: We evaluated the utility of this new method for the diagnosis of VWD in clinical practise.
Methods: Frozen plasma samples with known VWF:Activity and VWF:Ag levels of 88 consecutive patients having in-house VWF:MM as part of their evaluation for suspected VWD between 2017-2020 were available for study. The HYDRAGEL VWF:MM was performed using the 5- or 11 VWF multimer kits (Sebia, France). VWD diagnosis was based on the patient’s bleeding score (ISTH-BAT), VWF:Activity, VWF:Ag, factor VIII:C, multimer distribution and densitometry, and compared to the historic in-house VWF:MM and VWD diagnosis.
Results: Overall agreement of VWD diagnosis between the in-house and the automated VWF:MM was 74% (concordant in 65/88 patients, including 3 patients with undefined VWD), with VWD ruled out in 13 patients, VWD type 1 in 11, type 2A(IIA)/2B in 24, type 2A(IIE) in 29, type 2M in 2, type 2N in 1, and acquired Von Willebrand syndrome (AVWS) in 15 patients, respectively. Discrepant findings pertained primarily to VWD 2M and AVWS, which showed a large heterogeneity in the multimer distribution. Furthermore, three patients with defined VWD type 2A subtypes were not identified with HYDRAGEL VWF:MM, among them was a genetically confirmed VWD type 2A(IID). Results were available within one working day with the HYDRAGEL VWF:MM with implemented densitometry improved the interpretation and detection of slight structural variations that were not apparent by visual interpretation of the in-house VWF:MM.
Conclusions: HYDRAGEL VWF:MM analysis is a rapid, sensitive and standardized assay, which can be run as an initial VWF:MM screen when evaluating patients with a possible VWD.
To cite this abstract in AMA style:Brodard J, Rubin R, Baumgarnter D, Reusser M, A Kremer J, Hovinga . Utility of a New, Rapid Automated von Willebrand Factor (VWF) Multimer Assay for the Diagnosis of von Willebrand Disease [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/utility-of-a-new-rapid-automated-von-willebrand-factor-vwf-multimer-assay-for-the-diagnosis-of-von-willebrand-disease/. Accessed January 26, 2022.
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