脑苷脂网状内皮细胞病,又名原发性类脂质贮藏障碍病。本病为一少见的先天性、家族性类脂质代谢紊乱之疾病,多见于婴儿、小儿、青少年则极为少见。其病理特点为网状内皮系统如脾、骨髓、肝、淋巴结等处网状内皮细胞增生且代谢失常,呈现出脑苷脂沉积。该细胞首先由Gaucher氏于1885年描述,故又称Gaucher氏细胞,亦有人称高雪氏病。本病多见犹太人。在国内文献中,1948年钟惠兰等氏曾报告1例。患者为十五岁男性,但脾肿大已十年之久。张安氏曾见兄妹二人同患本病。迄今已有十余例。我院遇见一 Cerebroside Reticuloendothelial disease, also known as primary lipid storage disorders. The disease is a rare congenital, familial lipid disorders disorders, more common in infants, children, adolescents are extremely rare. The pathological features of reticuloendothelial system such as spleen, bone marrow, liver, lymph nodes and other reticuloendothelial hyperplasia and metabolic disorders, showing cerebroside deposition. The cell first described by Gaucher in 1885, it is also known as Gaucher’s cells, also known as Gaucher’s disease. The disease more common Jews. In the domestic literature, 1948 Zhong Whelan et al. Had reported 1 case. The patient was a fifteen-year-old man, but the splenomegaly had been ten years old. Zhang An’s brother and sister have seen two with the disease. So far there are more than ten cases. I met a hospital