唯支持细胞综合征病理变化和发病机制

The pathology and pathogenesis of sertoli cell only syndrome

目的:观察唯支持细胞综合征(SCOS)患者睾丸的病理变化,并探讨其可能的发病机制。方法:选取70例SCOS睾丸标本,通过光镜、电镜观察形态学改变,采用免疫组化方法分析其中40例石蜡标本中GATA-4及Inhibin-α的表达。并测定患者周围血激素水平。结果:①光镜下主要表现为曲细精管内生精细胞完全缺如,生精上皮仅由支持细胞组成,支持细胞显著增生,排列疏松、紊乱,部分曲细精管广泛纤维化、玻璃样变性、皱缩、塌陷,甚至管腔闭锁。间质细胞形态正常,相对增生。②电镜下主要表现为界膜胶原纤维增生,支持细胞胞浆内质网较丰富,有轻微扩张,部分线粒体嵴消失,甚至空泡变,支持细胞胞浆内出现较多自噬体。③40例SCOS睾丸标本免疫组化染色显示,GATA-4均呈阴性表达,Inhibin-α均呈阳性表达。④血清卵泡刺激素(FSH)值较正常成年男性水平升高,差异有统计学意义(P<0.01),而黄体生成素(LH)、睾酮(T)差异无统计学意义(P>0.05)。结论:支持细胞的线粒体病变及其功能受损是SCOS的病变基础,血清FSH浓度升高、GATA-4在支持细胞的表达缺失可能与SCOS的发生有关。

Objective： To observe the testicular pathological changes in sertoli cell only syndrome （SCOS） and investigate its pathogenesis. Methods： Testicular structure of seventy cases of SCOS was observed under light microscopy （LM） and transmission electron microscopy （TEM）. The hormone ieveis in the serum were detected with RIA, and the protein expressions of GATA-4 and Inhibin- α in the testis from 40 cases of SCOS were investigated with immunohistochemistry. Results： Under LM, it is manifestated as that there were only sertoii ceils within the seminiferous tubules,with proliferation and disordered arrangement in loose pattern, the seminiferous tubules showed fibrosis and hyalinization of the limiting membrane, shrinkage, collapse and atresia of the lumen, disappearance of germ cells and proliferation of Leydig cells. Under TEM, it is manifestated as proliferation of collagen fibers, abundance of cytoplasm endoplasmic reticulum with mild expansion in the sertoli cells, disappearance of mitochondrial cristae, even vacuolation of mitochondria, and autophagosomes in the cytoplasm of sertoli ceils. Immunohistochemically, Inhibin-α showed positive expression, while GATA-4 showed negative. The serum follicle stimulating hormone （FSH） levels were significantly higher than that of normal adult male, whereas luteinizing hormone （LH） and testosterone （T） were no significance compared with that of normal adult male. Conclusoin： The damage of mltochondrla and disturbance of the function of sertoli cell may be the basis of SCOS, the increase of FSH in the serum and loss of GATA-4 expression in sertoli cells may be involved in the pathogenesis of SCOS.