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VIII因子抑制剂的实验室评估:何时需要进行?基于当地实践的观点。

Laboratory Assessment of Factor VIII Inhibitors: When Is It Required? A Perspective Informed by Local Practice.

作者信息

Favaloro Emmanuel J, Curnow Jennifer, Pasalic Leonardo

机构信息

Haematology, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, NSW 2145, Australia.

School of Dentistry and Medical Sciences, Faculty of Science and Health, Charles Sturt University, Wagga, NSW 2650, Australia.

出版信息

J Clin Med. 2024 Dec 24;14(1):13. doi: 10.3390/jcm14010013.

Abstract

This perspective discusses the critical role of laboratory assessments in assessing factor VIII (FVIII) inhibitors. These are auto- and alloantibodies that can develop against both endogenous and exogenous FVIII, respectively. Assessment for inhibitors represents a key part of the management of both congenital hemophilia A (CHA), an inherited deficiency, and acquired hemophilia A (AHA), an autoimmune condition. Both conditions pose significant bleeding risks, necessitating careful monitoring of FVIII levels and inhibitor presence and level. Laboratory assays, particularly the Bethesda assay, are essential for detecting these inhibitors and assessing their levels. The complexities of FVIII inhibitor kinetics may pose challenges to interpretation of assay results, such that even normal FVIII levels do not always exclude inhibitor presence. Clinical practice guidelines recommend ongoing monitoring of AHA/CHA patients until inhibitors are no longer detectable. Overall, timely laboratory evaluations are essential to optimizing treatment strategies for patients with hemophilia, aiming to improve patient outcomes and quality of life. We summarize our approach to the laboratory assessment of FVIII inhibitors, as reflecting our perspective and as informed by local practice.

摘要

这篇综述讨论了实验室评估在评估凝血因子VIII(FVIII)抑制物中的关键作用。这些抑制物分别是可针对内源性和外源性FVIII产生的自身抗体和同种抗体。抑制物的评估是先天性A型血友病(CHA,一种遗传性缺乏症)和获得性A型血友病(AHA,一种自身免疫性疾病)管理的关键部分。这两种疾病都带来显著的出血风险,因此需要仔细监测FVIII水平以及抑制物的存在情况和水平。实验室检测,尤其是贝塞斯达检测,对于检测这些抑制物并评估其水平至关重要。FVIII抑制物动力学的复杂性可能给检测结果的解读带来挑战,以至于即使FVIII水平正常也不总是能排除抑制物的存在。临床实践指南建议持续监测AHA/CHA患者,直到检测不到抑制物为止。总体而言,及时的实验室评估对于优化血友病患者的治疗策略至关重要,旨在改善患者预后和生活质量。我们总结了我们对FVIII抑制物进行实验室评估的方法,以反映我们的观点并参考当地的实践经验。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66a0/11720995/fe854d924c59/jcm-14-00013-g001.jpg

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