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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...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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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. |
format | Online Article Text |
id | pubmed-8621170 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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