Review of laboratory methods used for analysis of von Willebrand factor and for diagnosis of related diseases.

IF 2.3 4区 医学 Q2 HEMATOLOGY
Jürgen Patzke, Nikolaus B Binder, Mariona Bono, François Depasse, Matthias Germer, Michael Heins, Martina Leplatoni, Stephan Schwers, Michael Spannagl, Peter L Turecek
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引用次数: 0

Abstract

Introduction: Von Willebrand factor (VWF) is a large multimeric protein present in the blood. Its most important and best characterized function is to control bleeding in primary hemostasis, which is triggered by different biophysical mechanisms and protein-receptor interactions involving different domains of VWF. Many different diseases related to VWF, most importantly von Willebrand disease comprising different types and sub-types, require diagnosis, laboratory analysis of concentration, and function of VWF.

Areas covered: As several different specific functions of VWF are physiologically relevant, several different assays are needed. The aim of this article is to provide a comprehensive overview of all relevant assays together with a description of how each assay is technically designed. Furthermore, guidance is given for choosing the right and validated assay methods.

Expert opinion: This information includes technical requirements, analytical performance data, references to relevant guidelines and other guidance documents, and reference standards. Also, information on availability and the type of the assay is provided, such as automated or manual, in vitro diagnostics or research use only. Relation to the clinical use of these assays, as well as performance, and result interpretation is covered in a second article in the same issue of this journal by the same authors.

血管性血友病因子分析及相关疾病诊断的实验室方法综述。
简介:血管性血友病因子(VWF)是一种存在于血液中的大型多聚体蛋白。它最重要和最具特色的功能是控制原发性止血中的出血,这是由不同的生物物理机制和涉及VWF不同结构域的蛋白质-受体相互作用引发的。许多与VWF相关的不同疾病,最重要的是由不同类型和亚型组成的血管性血友病,需要诊断、实验室分析VWF的浓度和功能。所涵盖的领域:由于VWF的几种不同的特定功能与生理相关,因此需要几种不同的测定方法。本文的目的是提供所有相关分析的全面概述,并描述每个分析是如何在技术上设计的。此外,指导选择正确和有效的分析方法。专家意见:这些信息包括技术要求、分析性能数据、对相关指南和其他指导文件的参考以及参考标准。此外,还提供了有关可用性和检测类型的信息,例如自动或手动、体外诊断或仅用于研究。与这些检测方法的临床应用以及性能和结果解释的关系,由同一作者在本刊同一期的第二篇文章中进行了介绍。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
4.70
自引率
3.60%
发文量
98
审稿时长
6-12 weeks
期刊介绍: Advanced molecular research techniques have transformed hematology in recent years. With improved understanding of hematologic diseases, we now have the opportunity to research and evaluate new biological therapies, new drugs and drug combinations, new treatment schedules and novel approaches including stem cell transplantation. We can also expect proteomics, molecular genetics and biomarker research to facilitate new diagnostic approaches and the identification of appropriate therapies. Further advances in our knowledge regarding the formation and function of blood cells and blood-forming tissues should ensue, and it will be a major challenge for hematologists to adopt these new paradigms and develop integrated strategies to define the best possible patient care. Expert Review of Hematology (1747-4086) puts these advances in context and explores how they will translate directly into clinical practice.
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