戈谢病,即葡糖脑苷脂病,是一种家族性糖脂代谢病,为常色体隐性遗传病[1]。其是由于酸性β-葡糖苷酶缺陷,造成葡糖脑苷脂沉积在单核巨噬细胞系统内形成形态特异的戈谢细胞,常累及骨髓、肝脏、脾脏及神经系统。随病情发展并发全血细胞减少、骨髓病变及神经系统症状等。我院1997~2 Gaucher disease, which is glucocerebrosidosis, is a familial glycolipid metabolic disease, a recessive genetic disease of the common-sense [1]. It is due to acid β-glucosidase deficiency, resulting in glucocerebrosidase deposition in the monocyte-macrophage system to form specific Goose cells, often involving the bone marrow, liver, spleen and nervous system. With the progression of concomitant pancytopenia, bone marrow disease and neurological symptoms. Our hospital 1997 ~ 2