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血小板功能检测在遗传性出血性疾病诊断中的现状与未来展望

Current status and future prospects for platelet function testing in the diagnosis of inherited bleeding disorders.

作者信息

Dovlatova Natalia

机构信息

Division of Clinical Neuroscience, Thrombosis and Haemostasis Research Group, Queens Medical Centre, University of Nottingham, Nottingham, UK.

出版信息

Br J Haematol. 2015 Jul;170(2):150-61. doi: 10.1111/bjh.13405. Epub 2015 Apr 28.

Abstract

Platelets play a crucial role in haemostasis by preventing bleeding at the site of vascular injury. Several defects in platelet morphology and function have been identified and described over the years. Although a range of methodologies is available to assess platelet function, a significant proportion of subjects with bleeding symptoms and normal coagulation parameters still appear to have normal results on platelet function testing. This might suggest that the reason for bleeding is multifactorial and is due to a combination of several minor defects in platelet function and/or other parts of the haemostatic system or might indicate that the currently available platelet function tests do not provide optimal diagnostic power. This review will summarize the established platelet function tests used for diagnosing inherited platelet abnormalities in adults and children, and discuss the newly developed methodologies as well as unmet challenges and potential areas for further improvement in this field.

摘要

血小板通过防止血管损伤部位出血在止血过程中发挥关键作用。多年来,人们已经识别并描述了血小板形态和功能方面的几种缺陷。尽管有一系列方法可用于评估血小板功能,但相当一部分有出血症状且凝血参数正常的受试者在血小板功能测试中似乎仍有正常结果。这可能表明出血原因是多因素的,是血小板功能和/或止血系统其他部分的几个微小缺陷共同作用的结果,或者可能表明目前可用的血小板功能测试没有提供最佳诊断能力。本综述将总结用于诊断成人和儿童遗传性血小板异常的既定血小板功能测试,并讨论新开发的方法以及该领域尚未满足的挑战和进一步改进的潜在领域。

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