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电子显微镜检查血小板整体铺片以定量血小板致密颗粒数量:对诊断由于致密颗粒缺乏引起的疑似血小板功能障碍的意义。

Electron microscopy examination of platelet whole mount preparations to quantitate platelet dense granule numbers: Implications for diagnosing suspected platelet function disorders due to dense granule deficiency.

机构信息

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

Centre for Laboratory Medicine and Hemophilia and Hemostasis Centre, St. Gallen, Switzerland.

出版信息

Int J Lab Hematol. 2018 Aug;40(4):400-407. doi: 10.1111/ijlh.12801. Epub 2018 Mar 6.

Abstract

INTRODUCTION

Dense granule (DG) deficiency (DGD) is a feature of some platelet function disorders (PFD) with a prevalence similar to von Willebrand disease. Most laboratories assess for DGD using whole mount platelet preparations and electron microscopy (EM). We evaluated our experiences with this test and associations between DGD and bleeding.

METHODS

Dense granule EM records for 2006-2017 were examined for patients and simultaneously tested controls, and for an overlapping PFD study cohort to evaluate findings and their relationship to bleeding.

RESULTS

More patient than control samples had reduced DG counts (6.5% vs 0.3%, P < .01). DG counts showed no relationship to age or mean platelet volume and had acceptable within-subject variability that was higher for DGD than control participants (28% vs 12%). Repeat tests confirmed DGD in all persons with initial DG counts <4.0/platelet, but not in those with less severe reductions (4.0-4.8 DG/platelet) or normal DG counts (≥4.9 DG/platelet). Aggregometry and adenosine triphosphate release tests, respectively, had only ~52% and 70% sensitivity for DGD. Confirmed DGD by EM was associated with higher bleeding scores and a bleeding disorder.

CONCLUSION

Whole mount EM is useful for the evaluation of suspected PFD due to DGD and detects abnormalities associated with bleeding.

摘要

简介

致密颗粒(DG)缺乏症(DGD)是某些血小板功能障碍(PFD)的特征,其患病率与 von Willebrand 病相似。大多数实验室使用血小板全片制备和电子显微镜(EM)来评估 DGD。我们评估了该测试的经验以及 DGD 与出血之间的关联。

方法

检查了 2006-2017 年患者和同时检测的对照者的致密颗粒 EM 记录,并对重叠的 PFD 研究队列进行了评估,以评估发现结果及其与出血的关系。

结果

与对照组相比,患者组的 DG 计数减少(6.5% vs 0.3%,P<.01)。DG 计数与年龄或平均血小板体积无关,且具有可接受的个体内变异性,DGD 参与者的变异性高于对照组(28% vs 12%)。重复测试证实了初始 DG 计数<4.0/血小板的所有患者的 DGD,但不能证实 DG 计数减少程度较轻(4.0-4.8 DG/血小板)或 DG 计数正常(≥4.9 DG/血小板)的患者的 DGD。聚集测定和三磷酸腺苷释放试验对 DGD 的敏感性分别约为 52%和 70%。通过 EM 证实的 DGD 与较高的出血评分和出血性疾病相关。

结论

血小板全片 EM 对于由于 DGD 引起的疑似 PFD 的评估是有用的,并且可以检测到与出血相关的异常。

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