Trung P H, Griscelli C, Mozziconacci P
Pathol Biol (Paris). 1975 Jan;23(1):57-62.
The platelets of 3 undoubted cases of Wiskott-Aldrich syndrome and of 1 suspected case have been investigated from the ultrastructural point of view. The platelet defects were striking by their small size, variable and distorted shape with numerous microvillosities, degranulated cytoplasm (scarce granules or lysosomes, rare mitochondria) and the distension of the canalicular system. The specificity of the ultrastructural platelet defects was discussed in comparison with other hereditary platelet diseases. An attempt was made to find out the meaning of the platelet alterations in this immunologic deficiency.
从超微结构角度对3例确诊的维斯科特-奥尔德里奇综合征患者及1例疑似患者的血小板进行了研究。血小板缺陷显著，表现为体积小、形状多变且扭曲，有大量微绒毛，细胞质脱颗粒（颗粒或溶酶体稀少，线粒体罕见）以及管道系统扩张。与其他遗传性血小板疾病相比，讨论了超微结构血小板缺陷的特异性。试图探究这种免疫缺陷中血小板改变的意义。
