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血管性血友病因子是止血过程中一个多面手,需要多种分析和诊断方法。

Von Willebrand factor is a multifaceted player in hemostasis requiring a diverse array of analytical and diagnostic approaches.

作者信息

Depasse François, Binder Nikolaus B, Bono Mariona, Germer Matthias, Heins Michael, Leplatoni Martina, Patzke Jürgen, Schwers Stephan, Spannagl Michael, Turecek Peter L

机构信息

Medical Affairs, Diagnostica Stago, Asnieres sur Seine, France.

Member of the Standardization Committee, Normenausschuss NA 176-08-04 AA Hämostaseologie, Deutsches Institut für Normung e.V. (DIN), Berlin, Germany.

出版信息

Expert Rev Hematol. 2025 Jul 9:1-18. doi: 10.1080/17474086.2025.2525458.

Abstract

INTRODUCTION

Von Willebrand factor (VWF) plays a crucial role in hemostasis: its interactions with endothelial matrices, platelets, and factor VIII make it a key player in both primary hemostasis and coagulation. Pathology associated with VWF spans mild to severe bleeding manifestations in the case of inherited von Willebrand disease (VWD), the most common congenital bleeding disorder, or acquired von Willebrand syndrome (AVWS). Conversely, VWF can be associated with thrombotic manifestations in situations related to inflammation, infection, or inherited or acquired ADAMTS13 defects.

AREAS COVERED

This review article aims to provide guidance on the use and interpretation of clinical laboratory assays available to measure VWF and other factors related to VWD. Different VWF tests can be used in different clinical settings for efficient diagnosis and patient management. Assay limitations are also addressed.

EXPERT OPINION

A myriad of laboratory assays, from first line to esoteric assays, exist to enable adequate diagnosis of VWD and other diseases influenced by VWF. Clinical investigations of VWF are complicated because VWF has multiple functions which variably depend on the patient's pathophysiological status. The right choice of assays is therefore critical to provide adequate diagnosis in due time and with reasonable analytical efforts.

摘要

引言

血管性血友病因子(VWF)在止血过程中起着至关重要的作用:它与内皮基质、血小板以及凝血因子 VIII 的相互作用使其成为原发性止血和凝血过程中的关键因素。与 VWF 相关的病理学表现涵盖了从轻度到重度的出血症状,如遗传性血管性血友病(VWD),这是最常见的先天性出血性疾病,以及获得性血管性血友病综合征(AVWS)。相反,在与炎症、感染或遗传性或获得性 ADAMTS13 缺陷相关的情况下,VWF 可能与血栓形成表现有关。

涵盖领域

本文旨在为临床实验室检测 VWF 及其他与 VWD 相关因子的使用和解读提供指导。不同的 VWF 检测可用于不同的临床场景,以实现高效诊断和患者管理。同时也讨论了检测的局限性。

专家观点

存在从一线检测到复杂检测等众多实验室检测方法,以充分诊断 VWD 和其他受 VWF 影响的疾病。对 VWF 的临床研究较为复杂,因为 VWF 具有多种功能,这些功能在不同程度上取决于患者的病理生理状态。因此,正确选择检测方法对于及时、合理地进行充分诊断至关重要。

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