ArticleResearch and practice in thrombosis and haemostasis2020
Improving diagnosis of von Willebrand disease: Reference ranges for von Willebrand factor multimer distribution.
Article in Research and practice in thrombosis and haemostasis, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed.
- Landscape and Spectrum of VWF Variants in Type 2 Von Willebrand Disease: Insights from a German Patient Cohort.Thrombosis and haemostasis · 2026Article
- Ratio of Von Willebrand Collagen Binding Assay and Von Willebrand Antigen Can Predict Multimer Size in Von Willebrand Disease.Haemophilia : the official journal of the World Federation of Hemophilia · 2025Article
- Clinical utility of panel-based genetic sequencing for von Willebrand disease.Research and practice in thrombosis and haemostasis · 2025Article
- Analysis of von Willebrand Disease in the "Heart of Europe".TH open : companion journal to thrombosis and haemostasis · 2022Article
- Von Willebrand Factor Multimer Analysis by Low Resolution SDS-Agarose Gel Electrophoresis.Bio-protocol · 2022Article
- Establishing reference intervals for von Willebrand factor multimers.Journal of medical biochemistry · 2022Article
- Multimer Analysis of Von Willebrand Factor in Von Willebrand Disease with a Hydrasys Semi-Automatic Analyzer-Single-Center Experience.Diagnostics (Basel, Switzerland) · 2021Article
- Dynamic Effects in Nucleation of Receptor Clusters.Entropy (Basel, Switzerland) · 2021Article
- Resolving Differential Diagnostic Problems in von Willebrand Disease, in Fibrinogen Disorders, in Prekallikrein Deficiency and in Hereditary Hemorrhagic Telangiectasia by Next-Generation Sequencing.Life (Basel, Switzerland) · 2021Article
- Type 2A von Willebrand disease and systemic sclerosis: Vonicog alfa reduced gastrointestinal bleeding.Research and practice in thrombosis and haemostasis · 2020Article
- Improving diagnosis of von Willebrand disease: Reference ranges for von Willebrand factor multimer distribution.Research and practice in thrombosis and haemostasis · 2020Article
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2 authors.
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Abstract
backgroundPhenotypic von Willebrand disease (VWD) classification requires multiple tests including analysis of multimeric distributions von Willebrand factor (VWF) and evaluation of its structure. VWF multimer analysis is labor intensive, nonstandardized, and limited to specialized laboratories. A commercial semiautomatic assay, HYDRAGEL VW multimer assay (H5/11VWM, Sebia), has become available.
objectivesEstablishment of reference ranges for H5/11VWM to improve VWD classification.
methodsImplementation validation, establishment and validation of normal and pathological reference intervals (NRIs/PRIs), comparison with in-house method using 40 healthy volunteers and 231 VWD patients.
resultsQualitative and quantitative validation of NRI obtained sensitivity of 88% and 79%, respectively, for type 2. Comparison of the two methods showed an overall concordance of 86% with major conflicting results in all atypical 2B (n = 7) and 50% 2M-GPIb (n = 41) showing quantitative and qualitative multimeric loss, that was not detected with in-house method. We were able to use established PRIs, with 73% validity in type 2 cases, to distinguish individual type 2A subtypes (IIA, IIC, IID, IIE) from 2M and 2B.
conclusionH5/11VWM could be used for all clinical purposes because its reliability and its rapid and accurate diagnostic ability and reduced observer bias. Although H5/11VWM cannot evaluate triplet structures, we were able to define 2A subtypes by stripping back to the percentage of intermediate/high-molecular-weight multimers. H5/11HWM could be an efficient and widely available alternative for the "gold standard" technique.
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