高雪氏病是一种较少見的常染色体隐性遗传的代谢性疾病,由于缺乏葡萄糖脑甙酶,致葡萄糖脑甙在单核—巨细胞系统内沉积而致病。我国从1948年迄今已报导约30例,本文报告我院所见6例,均经骨髓、脾、淋巴结穿刺,涂片瑞氏—吉姆沙染色,Mallory氏染色,PAS染色而确诊。 Gaucher’s disease is a rare autosomal recessive metabolic disease that is caused by the deposition of glucoside in the mononuclear-giant cell system due to the lack of glucosidase. So far, about 30 cases have been reported in our country from 1948 to the present. Six cases in our hospital were reported in this paper. All of them were diagnosed by bone marrow, spleen, lymph node puncture, smear Wright-Giemsa staining, Mallory’s staining and PAS staining.