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血小板功能检测:北欧临床中心的当前实践。

Platelet function testing: Current practice among clinical centres in Northern Europe.

机构信息

Coagulation Disorders Unit, Department of Hematology, Comprehensive Cancer Center, Helsinki University Hospital, Helsinki, Finland.

Research Program in Systems Oncology, Helsinki University, Helsinki, Finland.

出版信息

Haemophilia. 2022 Jul;28(4):642-648. doi: 10.1111/hae.14578. Epub 2022 May 5.

Abstract

INTRODUCTION

Platelet function tests are used to screen and diagnose patients with possible inherited platelet function defects (IPFD). Some acquired platelet dysfunction may be caused by certain drugs or comorbidities, which need to be excluded before testing.

AIMS

To identify current practice among centres performing platelet function tests in Northern Europe.

METHODS

A total of 14 clinical centres from Sweden (six), Finland (two), Denmark (two), Norway (one), Estonia (two) and Iceland (one) completed the survey questionnaire, the population capture area of about 29.5 million.

RESULTS

Six of the 14 (42.8%) centres providing platelet function assessment represent comprehensive treatment centres (EUHANET status). A Bleeding score (BS) or ISTH bleeding assessment tool (ISTH BAT score) is evaluated in 11/14 (78.6%) centres and family history in all. Five/14 centres (35.7%) use structured preanalytical patient instructions, and 10/14 (71.4%) recorded questionnaire on the preassessment of avoidance of any drugs or natural products affecting platelet functions. Preliminary investigations of screening tests of coagulation are performed in 10/14 (71.4%), while in 4/14 (28.6%), the diagnostic work-up of IPFD and von Willebrand disease (VWD) is performed simultaneously. The work-up of IPFD includes peripheral blood smear in 10/14 (71.4%), platelet aggregometry in all, flow cytometry in 10/14 (71.4%) and Platelet Function Analysis (PFA) in 3/11 (28.6%). Molecular genetic diagnosis is available in 7/14 (50%) centres.

CONCLUSIONS

The considerable variability in the current practice illustrates the need for harmonization between the Northern European centres according to the international registers (i.e. EUHASS) and IPFD guidelines (ISTH, EHA).

摘要

简介

血小板功能检测用于筛查和诊断可能存在遗传性血小板功能缺陷(IPFD)的患者。某些获得性血小板功能障碍可能由某些药物或合并症引起,在进行检测之前需要排除这些因素。

目的

确定北欧进行血小板功能检测的中心的当前实践情况。

方法

来自瑞典(6 个)、芬兰(2 个)、丹麦(2 个)、挪威(1 个)、爱沙尼亚(2 个)和冰岛(1 个)的 14 个临床中心完成了问卷调查,涵盖约 2950 万人口。

结果

14 个提供血小板功能评估的中心中有 6 个(42.8%)是综合治疗中心(EUHANET 状态)。11/14(78.6%)的中心评估出血评分(BS)或 ISTH 出血评估工具(ISTH BAT 评分),所有中心都评估家族史。5/14(35.7%)中心使用结构化的预分析患者说明,10/14(71.4%)记录关于避免任何影响血小板功能的药物或天然产物的预评估问卷。10/14(71.4%)中心进行了初步凝血筛查检测,而在 4/14(28.6%)中心,同时进行了 IPFD 和血管性血友病(VWD)的诊断性检查。IPFD 诊断工作包括外周血涂片 10/14(71.4%)、全血小板聚集试验 14/14(100%)、流式细胞术 10/14(71.4%)和血小板功能分析(PFA)3/11(28.6%)。7/14(50%)中心可进行分子遗传学诊断。

结论

当前实践中的显著差异表明北欧中心需要根据国际登记册(即 EUHASS)和 IPFD 指南(ISTH、EHA)进行协调。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b938/9540416/6e9dbe28df4d/HAE-28-642-g003.jpg

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