流式细胞术在检测血小板无力症中的意义

Significance of Flow Cytometry in Detection of Glanzmann's Thrombasthenia

血小板上含有丰富的GPⅡ-Ⅲa,在血小板聚集时,GPⅡb-Ⅲa复合物上的纤维蛋白原受体与纤维蛋白原结合。血小板无力症(Glanzmann’s thrombasthenia,GT)患者是GPⅡb-Ⅲa复合物的质或量的缺陷所致血小板聚集异常。通常用聚丙烯酸胺凝胶电泳(SDS-PAGE)及Western印迹等方法检测膜糖蛋白,但操作烦琐、费时费试剂。流式细胞术(FCM)及特异性单机的应用使血小板膜GP检测有了新突破,特别是纤维蛋白原结合试验使变异型GT的诊断成为可能。我们用FCM检测12例GT患者及其家属的血小板膜GP及纤维蛋白原结合并与SDS-PAGE和Western印迹做比较,以探索FCM方法对GT诊断的价值和意义。

In order to detect fibrinogen binding to platelet and the platelet membrane glycoproteins (GP) expression in patients with Glanzmann's thrombasthenia (GT) by flow cytometry (FCM), and to ensure its value in GT diagnosis, 12 GT patients, their relatives (father or mother) and 12 normal individuals were involved in the study to analyse the platelet membrane GP Ⅰ b, Ⅱb, Ⅲ a and fibrinogen binding with FCM. The platelet membrane glycoproteins were also measured by SDS-PAGE and Western blot. The results showed that on FCM, the fibrinogen binding to platelet in all patients was lower than that of normal subjects, the GP Ⅱ b and Ⅲ a expression was remarkably decreased in 10 cases and relatively lowered in other 2 cases, while the GP Ⅰ b was normal in all patients. Similar results were seen in detections with PAGE and Western blot. The results suggest that measurement of fibrinogen binding and platelet membrane GP expression by FCM in whole blood can provide a rapid, sensitive and simple method for GT diagnosis.