本文报道１例Ｇａｕｃｈｅｒ病及１例Ｎｉｅｍａｎｎ－Ｐｉｃｋ病的组织学和超微结构形态改变。光镜下Ｇａｕｃｈｅｒ细胞及Ｎｉｅｍａｎｎ－Ｐｉｃｋ细胞都为泡沫样细胞，形态类似。但透射电镜下两种细胞形态完全不同，Ｇａｕｃｈｅｒ细胞内出现较多大囊泡，而Ｎｉｅｍａｎｎ－Ｐｉｃｋ细胞内则出现大量特征性的板层小体。因此，电镜对这两种疾病的鉴别诊断具有重要作用。 This article reports the histological and ultrastructural changes of one case of Gaucher disease and one case of Niemann-Pick disease. Under light microscope Gaucher cells and Niemann-Pick cells are foam-like cells, similar in morphology. However, under transmission electron microscopy, the morphology of the two cells was completely different. There were many large vesicles in Gaucher cells, while a large number of lamellar bodies appeared in Niemann-Pick cells. Therefore, electron microscopy in the differential diagnosis of these two diseases has an important role.