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探讨常见出血性疾病的方法:对北美凝血实验室实践的评估。

Approaches to investigating common bleeding disorders: an evaluation of North American coagulation laboratory practices.

机构信息

Department of Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada.

出版信息

Am J Hematol. 2012 May;87 Suppl 1:S45-50. doi: 10.1002/ajh.23124. Epub 2012 Feb 24.

DOI:10.1002/ajh.23124
PMID:22367923
Abstract

Bleeding disorders commonly result from deficiencies or defects in von Willebrand factor (VWF), platelets, coagulation factors, or fibrinolytic proteins. The primary goal of our study was to assess current North American coagulation laboratory practices for diagnosing bleeding disorders, using an on-line patterns-of-practice survey of diagnostic laboratory members of the North American Specialized Coagulation Laboratory Association. The survey examined laboratory approaches to evaluating bleeding disorders, with specific questions about the tests and test panels offered and compliance to recent guideline recommendations on diagnosing von Willebrand disease (VWD) and platelet function disorders. All laboratories responding to the survey performed a prothrombin time/international normalized ratio, an activated partial thromboplastin time, and coagulation factor assays, and many tested for VWD and platelet disorders. However, few laboratories had test panels that evaluated the more common bleeding disorders and few performed some assays, including VWF multimer assessments and assays for fibrinolytic disorders. Additionally, the cutoffs used by laboratories to diagnose type 1 VWD varied considerably, with only a minority following the National Heart Lung Blood Institute recommendations. In contrast, laboratories that tested for platelet function disorders mostly complied with aggregation testing recommendations, as published in the recent North American guidelines. Our results indicate that there are some gaps in the strategies used by laboratories to diagnose bleeding disorders that might be addressed by development of further guidelines and test algorithms that emphasize evaluations for common bleeding disorders. Laboratories may also benefit from guidelines on test interpretation, and external evaluation of their bleeding disorder testing strategies.

摘要

出血性疾病通常是由于血管性血友病因子 (VWF)、血小板、凝血因子或纤维蛋白溶解蛋白的缺乏或缺陷引起的。我们的主要研究目标是使用北美专门凝血实验室协会的诊断实验室成员在线实践模式调查,评估北美当前的凝血实验室诊断出血性疾病的实践。该调查研究了评估出血性疾病的实验室方法,具体问题涉及提供的测试和测试组合以及最近关于诊断血管性血友病 (VWD) 和血小板功能障碍的指南建议的遵守情况。对调查做出回应的所有实验室都进行了凝血酶原时间/国际标准化比值、活化部分凝血活酶时间和凝血因子测定,并且许多实验室都检测了 VWD 和血小板疾病。然而,很少有实验室有评估常见出血性疾病的测试组合,也很少进行一些检测,包括 VWF 多聚体评估和纤维蛋白溶解障碍检测。此外,实验室用于诊断 1 型 VWD 的截止值差异很大,只有少数实验室遵循国家心肺血液研究所的建议。相比之下,测试血小板功能障碍的实验室大多遵守最近的北美指南中公布的聚集测试建议。我们的结果表明,实验室用于诊断出血性疾病的策略存在一些差距,这可能需要进一步制定指南和测试算法来强调对常见出血性疾病的评估来解决。实验室还可以从测试解释指南和对其出血性疾病测试策略的外部评估中受益。

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