Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience

von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder. This disorder develops as a result of defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for VWF-related disorders requires the assessment of both VWF level and VWF ac...

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Autores principales:	Ingrid Skornova, Tomas Simurda, Jan Stasko, Jana Zolkova, Juraj Sokol, Pavol Holly, Miroslava Dobrotova, Ivana Plamenova, Jan Hudecek, Monika Brunclikova, Alena Stryckova, Peter Kubisz
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	von Willebrand factor von Willebrand factor multimers von Willebrand disease electrophoresis diagnostics high-molecular weight multimers (HMW) Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/044a479f87c64ae6989a805ed7e053f0
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id	oai:doaj.org-article:044a479f87c64ae6989a805ed7e053f0
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spelling	oai:doaj.org-article:044a479f87c64ae6989a805ed7e053f02021-11-25T17:22:05ZMultimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience10.3390/diagnostics111121532075-4418https://doaj.org/article/044a479f87c64ae6989a805ed7e053f02021-11-01T00:00:00Zhttps://www.mdpi.com/2075-4418/11/11/2153https://doaj.org/toc/2075-4418von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder. This disorder develops as a result of defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for VWF-related disorders requires the assessment of both VWF level and VWF activity, the latter requiring multiple assays. As an additional step, an evaluation of VWF structural features by multimer analysis is useful in selective investigations. Multimer analysis is also important for the selection of a suitable VWF therapy preparation (desmopressin, VWF/FVIII concentrate, recombinant VWF) and the determination of the correct dose for the patient. Based on clinical and laboratory findings, including the analysis of VWF multimers, we classified our patients into individual types of VWD. Our study group included 58 patients. The study group consisted of 66% (38 patients) with VWD type 1, 5% (3 patients) with VWD type 2, 7% (4 patients) with VWD type 3, 5% (3 patients) with mixed type 1/2A VWD, and 17% (10 patients) comprising an unclassified group. In this article, we provide an overview of our practical experience using a new complementary method—the analysis of von Willebrand factor multimers with a semi-automatic analyzer Hydrasys 2 scan. We explain the principle, procedure, advantages, and pitfalls associated with the introduction of the VWF multimer analysis methodology into standard VWD diagnostics.Ingrid SkornovaTomas SimurdaJan StaskoJana ZolkovaJuraj SokolPavol HollyMiroslava DobrotovaIvana PlamenovaJan HudecekMonika BrunclikovaAlena StryckovaPeter KubiszMDPI AGarticlevon Willebrand factorvon Willebrand factor multimersvon Willebrand diseaseelectrophoresisdiagnosticshigh-molecular weight multimers (HMW)Medicine (General)R5-920ENDiagnostics, Vol 11, Iss 2153, p 2153 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	von Willebrand factor von Willebrand factor multimers von Willebrand disease electrophoresis diagnostics high-molecular weight multimers (HMW) Medicine (General) R5-920
spellingShingle	von Willebrand factor von Willebrand factor multimers von Willebrand disease electrophoresis diagnostics high-molecular weight multimers (HMW) Medicine (General) R5-920 Ingrid Skornova Tomas Simurda Jan Stasko Jana Zolkova Juraj Sokol Pavol Holly Miroslava Dobrotova Ivana Plamenova Jan Hudecek Monika Brunclikova Alena Stryckova Peter Kubisz Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience
description	von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder. This disorder develops as a result of defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for VWF-related disorders requires the assessment of both VWF level and VWF activity, the latter requiring multiple assays. As an additional step, an evaluation of VWF structural features by multimer analysis is useful in selective investigations. Multimer analysis is also important for the selection of a suitable VWF therapy preparation (desmopressin, VWF/FVIII concentrate, recombinant VWF) and the determination of the correct dose for the patient. Based on clinical and laboratory findings, including the analysis of VWF multimers, we classified our patients into individual types of VWD. Our study group included 58 patients. The study group consisted of 66% (38 patients) with VWD type 1, 5% (3 patients) with VWD type 2, 7% (4 patients) with VWD type 3, 5% (3 patients) with mixed type 1/2A VWD, and 17% (10 patients) comprising an unclassified group. In this article, we provide an overview of our practical experience using a new complementary method—the analysis of von Willebrand factor multimers with a semi-automatic analyzer Hydrasys 2 scan. We explain the principle, procedure, advantages, and pitfalls associated with the introduction of the VWF multimer analysis methodology into standard VWD diagnostics.
format	article
author	Ingrid Skornova Tomas Simurda Jan Stasko Jana Zolkova Juraj Sokol Pavol Holly Miroslava Dobrotova Ivana Plamenova Jan Hudecek Monika Brunclikova Alena Stryckova Peter Kubisz
author_facet	Ingrid Skornova Tomas Simurda Jan Stasko Jana Zolkova Juraj Sokol Pavol Holly Miroslava Dobrotova Ivana Plamenova Jan Hudecek Monika Brunclikova Alena Stryckova Peter Kubisz
author_sort	Ingrid Skornova
title	Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience
title_short	Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience
title_full	Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience
title_fullStr	Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience
title_full_unstemmed	Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience
title_sort	multimer analysis of von willebrand factor in von willebrand disease with a hydrasys semi-automatic analyzer—single-center experience
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/044a479f87c64ae6989a805ed7e053f0
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Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience

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