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CC BY-NC-ND 4.0 · J Lab Physicians 2021; 13(03): 195-201
DOI: 10.1055/s-0041-1730818
Original Article

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

Marika Pikta 
1   Department of Health Technologies, Tallinn University of Technology, Tallinn, Estonia
2   Department of Laboratory Medicine, North Estonia Medical Centre, Tallinn, Estonia
,
Valdas Banys
3   Department of Physiology, Biochemistry, Microbiology and Laboratory Medicine, Faculty of Medicine, Institute of Biomedical Sciences, Vilnius University, Vilnius, Lithuania
,
Timea Szanto
4   Research Program Unit of Systems Oncology, Helsinki University Hospital, Unit of Coagulation Disorders, Helsinki, Finland
,
Lotta Joutsi-Korhonen
5   Department of Clinical Chemistry, Unit of Coagulation Disorders, HUSLAB Laboratory Services, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
,
Ines Vaide
6   Hemato-Oncology Clinic, Institute of Clinical Medicine, Tartu University, Tartu, Estonia
,
Mirja Varik
7   Hematology Department, North Estonia Medical Centre, Tallinn, Estonia
,
Anna-Elina Lehtinen
8   Department of Hematology, Unit of Coagulation Disorders, Helsinki University Hospital Comprehensive Cancer Center and University of Helsinki, Helsinki, Finland
,
Paul Giangrande
9   Green Templeton College, University of Oxford, Oxford, United Kingdom
,
Edward Laane
6   Hemato-Oncology Clinic, Institute of Clinical Medicine, Tartu University, Tartu, Estonia
› Author Affiliations Research Funding Sebia (Lisses, France) has donated the Hydragel 5 von Willebrand multimers kits.
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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.

Keywords

acquired von Willebrand syndrome - von Willebrand factor - von Willebrand factor multimers

Note: The study was performed according to the Declaration of Helsinki and was approved by the Tallinn Medical Research Ethics Committee.




Publication History

Article published online:
28 June 2021

© 2021. The Indian Association of Laboratory Physicians. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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