Coller B S, Zarrabi M H
Blood. 1981 Aug;58(2):279-84.
Since studies of the giant platelets in the Bernard-Soulier syndrome have shown decreased electrophoretic mobility, decreased sialic acid, and an abnormality in a membrane glycoprotein, we performed similar studies on the giant platelets from two patients with the May-Hegglin anomaly. The patients' platelet electrophoretic mobilities did not differ from control. Although the total sialic acid contents of the patients' platelets were greater than control when calculated per platelet, they were very similar to control when normalized for differences in platelet volume and surface area. When platelet proteins were separated by sodium dodecyl sulfate polyacrylamide gel electrophoresis there were no differences between the glycoproteins of control and patient platelets as judged by the patterns of periodic acid Schiff staining and fluorescein-labeled concanavalin A binding. Similarly, patterns of surface glycoprotein labeling by neuraminidase/galactose oxidase/KB3H4 were identical. We conclude that unlike the giant platelets in the Bernard-Soulier syndrome, those of the May-Hegglin anomaly are not associated with a membrane abnormality detectable by these techniques.
由于对伯纳德-苏利耶综合征中巨型血小板的研究显示其电泳迁移率降低、唾液酸减少以及膜糖蛋白存在异常，我们对两名患有梅-赫格利恩异常的患者的巨型血小板进行了类似研究。患者血小板的电泳迁移率与对照组无差异。尽管按每个血小板计算，患者血小板的总唾液酸含量高于对照组，但在根据血小板体积和表面积差异进行标准化后，它们与对照组非常相似。当通过十二烷基硫酸钠聚丙烯酰胺凝胶电泳分离血小板蛋白时，根据高碘酸席夫染色模式和荧光素标记的伴刀豆球蛋白A结合判断，对照组和患者血小板的糖蛋白之间没有差异。同样，神经氨酸酶/半乳糖氧化酶/KB3H4对表面糖蛋白的标记模式也是相同的。我们得出结论，与伯纳德-苏利耶综合征中的巨型血小板不同，梅-赫格利恩异常的巨型血小板与这些技术可检测到的膜异常无关。
