Mori K, Suzuki S, Sugai K
Tohoku J Exp Med. 1984 Jul;143(3):261-87. doi: 10.1620/tjem.143.261.
The ultrastructure and functions of platelets and megakaryocytes in 8 patients of a family with gray platelet syndrome were investigated. Hemostatic examinations on these patients revealed prolonged bleeding time, decreased platelet retention rates and decreased platelet aggregation rates induced by ADP, collagen, Ristocetin and epinephrine. Marked decreases in ATP and ADP release in response to these agents were also noted. Clinical and coagulation studies on this family suggested that the hereditary nature of the syndrome is autosomal dominant. Platelets and megakaryocytes in the peripheral and bone marrow blood smear from the patients showed peculiar gray color by May-Giemsa stain due to a deficiency of alpha-granules. Electron microscopic examinations revealed slightly enlarged platelets containing a deficient amount of alpha-granules, whereas dense bodies and mitochondria appeared normal. Several morphological abnormalities of patient's platelets, such as aggregates of dense tubular systems, circular arrays of dense tubular systems, an area of cytoplasmic sequestration with an enclosing membrane, clumps of dense material and remnants of Golgi apparatuses were recognized. Megakaryocytes showed normally developed Golgi zones, defective alpha-granule synthesis and liberation of abnormal platelets as shown in the peripheral blood smear.
对一个患有灰色血小板综合征家族的8名患者的血小板和巨核细胞的超微结构及功能进行了研究。对这些患者的止血检查显示出血时间延长，血小板滞留率降低，以及由ADP、胶原、瑞斯托霉素和肾上腺素诱导的血小板聚集率降低。还观察到对这些试剂的ATP和ADP释放明显减少。对该家族的临床和凝血研究表明，该综合征的遗传性质为常染色体显性遗传。患者外周血和骨髓血涂片的血小板和巨核细胞经May-Giemsa染色后呈现特殊的灰色，这是由于α颗粒缺乏所致。电子显微镜检查显示血小板略有增大，α颗粒数量不足，而致密体和线粒体看起来正常。还识别出患者血小板的一些形态学异常，如致密管状系统聚集、致密管状系统呈环状排列、有包膜的细胞质隔离区、致密物质团块和高尔基体残余物。巨核细胞显示高尔基体区域发育正常，α颗粒合成缺陷，并且如外周血涂片所示异常血小板释放。
