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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: Skornova, Ingrid, Simurda, Tomas, Stasko, Jan, Zolkova, Jana, Sokol, Juraj, Holly, Pavol, Dobrotova, Miroslava, Plamenova, Ivana, Hudecek, Jan, Brunclikova, Monika, Stryckova, Alena, Kubisz, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8621170/
https://www.ncbi.nlm.nih.gov/pubmed/34829500
http://dx.doi.org/10.3390/diagnostics11112153
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author Skornova, Ingrid
Simurda, Tomas
Stasko, Jan
Zolkova, Jana
Sokol, Juraj
Holly, Pavol
Dobrotova, Miroslava
Plamenova, Ivana
Hudecek, Jan
Brunclikova, Monika
Stryckova, Alena
Kubisz, Peter
author_facet Skornova, Ingrid
Simurda, Tomas
Stasko, Jan
Zolkova, Jana
Sokol, Juraj
Holly, Pavol
Dobrotova, Miroslava
Plamenova, Ivana
Hudecek, Jan
Brunclikova, Monika
Stryckova, Alena
Kubisz, Peter
author_sort Skornova, Ingrid
collection PubMed
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.
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spelling pubmed-86211702021-11-27 Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience Skornova, Ingrid Simurda, Tomas Stasko, Jan Zolkova, Jana Sokol, Juraj Holly, Pavol Dobrotova, Miroslava Plamenova, Ivana Hudecek, Jan Brunclikova, Monika Stryckova, Alena Kubisz, Peter Diagnostics (Basel) Article 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. MDPI 2021-11-20 /pmc/articles/PMC8621170/ /pubmed/34829500 http://dx.doi.org/10.3390/diagnostics11112153 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Skornova, Ingrid
Simurda, Tomas
Stasko, Jan
Zolkova, Jana
Sokol, Juraj
Holly, Pavol
Dobrotova, Miroslava
Plamenova, Ivana
Hudecek, Jan
Brunclikova, Monika
Stryckova, Alena
Kubisz, Peter
Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer—Single-Center Experience
title 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_short 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
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8621170/
https://www.ncbi.nlm.nih.gov/pubmed/34829500
http://dx.doi.org/10.3390/diagnostics11112153
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