Von Willebrand因素与疾病:实验室专业人员综述。

IF 6.6 2区 医学 Q1 MEDICAL LABORATORY TECHNOLOGY Critical reviews in clinical laboratory sciences Pub Date : 2022-06-01 Epub Date: 2021-12-28 DOI:10.1080/10408363.2021.2014781
Neil S Harris, J Peter Pelletier, Maximo J Marin, William E Winter
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引用次数: 5

摘要

鉴于血管性血友病(VWD)是最常见的出血性疾病之一,在有不明原因出血的个体的检查中诊断或排除是必不可少的。对于临床实验室来说,这种疾病的不同表现和不同供应商提供的多种检测方法突出了挑战。本文将简要介绍原发性止血,并详细解释血管性血友病因子(VWF)的生物合成、结构和机制。最后几节将重点介绍不同类型VWD的区别特征以及目前可用于协助诊断的一系列临床实验室检查。
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Von Willebrand factor and disease: a review for laboratory professionals.

Given that von Willebrand disease (VWD) is one of the most common bleeding disorders, the diagnosis or the exclusion is essential in the workup of individuals that have unexplained bleeding. For the clinical laboratory, the challenge is highlighted by the variable presentations of this disorder and the multiple assays that are available from different vendors. This review will give a brief overview of primary hemostasis with a detailed explanation of the biosynthesis, structure, and mechanics of von Willebrand factor (VWF). The final sections will focus on the distinguishing characteristics of the different types of VWD and the array of clinical laboratory tests currently available to assist in the diagnosis.

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来源期刊
CiteScore
20.00
自引率
0.00%
发文量
25
审稿时长
>12 weeks
期刊介绍: Critical Reviews in Clinical Laboratory Sciences publishes comprehensive and high quality review articles in all areas of clinical laboratory science, including clinical biochemistry, hematology, microbiology, pathology, transfusion medicine, genetics, immunology and molecular diagnostics. The reviews critically evaluate the status of current issues in the selected areas, with a focus on clinical laboratory diagnostics and latest advances. The adjective “critical” implies a balanced synthesis of results and conclusions that are frequently contradictory and controversial.
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