Perutelli P, Marchese P, Mori P G
Department of Pediatric Hematology and Oncology, G. Gaslini Children's Hospital, Genoa, Italy.
Haemostasis. 1992;22(6):330-3. doi: 10.1159/000216343.
Glanzmann's thrombasthenia is a rare hemorrhagic syndrome, characterized by a quantitative or functional defect of the platelet glycoprotein GPIIb-IIIa complex. The authors describe a method to diagnose thrombasthenic patients and identify carrier subjects by using monoclonal antibodies specific for GPIIb and GPIIIa in an immunoblotting technique. The immunoreaction patterns of two thrombasthenic patients lacking GPIIb or GPIIIa, respectively, are shown. The described method produces further evidence concerning the biochemical heterogeneity of Glanzmann's thrombasthenia.
血小板无力症是一种罕见的出血综合征,其特征为血小板糖蛋白GPIIb-IIIa复合物存在数量或功能缺陷。作者描述了一种利用免疫印迹技术中针对GPIIb和GPIIIa的单克隆抗体来诊断血小板无力症患者并识别携带者的方法。展示了分别缺乏GPIIb或GPIIIa的两名血小板无力症患者的免疫反应模式。所描述的方法为血小板无力症的生化异质性提供了进一步的证据。