Von Willebrand Factor Multimeric Assay in Acquired von Willebrand Disease Diagnosis : A Report of Experience from North Estonia Medical Centre

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Pikta , M , Banys , V , Szanto , T , Joutsi-Korhonen , L , Vaide , I , Varik , M , Lehtinen , A-E , Giangrande , P & Laane , E 2021 , ' Von Willebrand Factor Multimeric Assay in Acquired von Willebrand Disease Diagnosis : A Report of Experience from North Estonia Medical Centre ' , Journal of laboratory physicians , vol. 13 , no. 03 , pp. 195-201 . https://doi.org/10.1055/s-0041-1730818

Title: Von Willebrand Factor Multimeric Assay in Acquired von Willebrand Disease Diagnosis : A Report of Experience from North Estonia Medical Centre
Author: Pikta, Marika; Banys, Valdas; Szanto, Timea; Joutsi-Korhonen, Lotta; Vaide, Ines; Varik, Mirja; Lehtinen, Anna-Elina; Giangrande, Paul; Laane, Edward
Contributor organization: HUSLAB
Clinicum
Research Program in Systems Oncology
Department of Clinical Chemistry and Hematology
University of Helsinki
Hematologian yksikkö
HUS Comprehensive Cancer Center
Date: 2021-09
Language: eng
Number of pages: 7
Belongs to series: Journal of laboratory physicians
ISSN: 0974-2727
DOI: https://doi.org/10.1055/s-0041-1730818
URI: http://hdl.handle.net/10138/340485
Abstract: Objectives Acquired von Willebrand syndrome (AVWS) is a rare and frequently underdiagnosed bleeding disorder with an unknown prevalence. The diagnosis of AVWS is made based on laboratory investigations and the presence of clinical symptoms. Evaluation and management of affected patients are complex due to the need for multiple laboratory assays. Materials and Methods Here, we describe the clinical and laboratory data of seven patients with a diagnosis of AVWS. All patients met the criteria for AVWS based on laboratory findings, bleeding symptoms, and the absence of any previous history of a bleeding disorder. Results In all cases, the laboratory findings, lack of bleeding anamnesis, and family history suggested the presence of AVWS. Von Willebrand factor multimeric analysis showed decreased high-molecular weight (HMW) multimers in six cases. Patients with lower HMW multimers experienced more severe bleeding complications. Conclusions The diagnosis of AVWS is complex and requires extensive laboratory evaluation. Interdisciplinary collaboration and complex laboratory evaluations are of paramount importance for the early recognition of AVWS and optimal AVWS diagnosis as well as successful clinical management.
Subject: acquired von Willebrand syndrome
von Willebrand factor
von Willebrand factor multimers
ESSENTIAL THROMBOCYTHEMIA
PATHOPHYSIOLOGY
CLASSIFICATION
3121 General medicine, internal medicine and other clinical medicine
Peer reviewed: Yes
Rights: cc_by_nc_nd
Usage restriction: openAccess
Self-archived version: publishedVersion


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