Iwai M, Kashiwadani M, Okuno T, Takino T, Koshikawa T
Third Department of Internal Medicine, Kyoto Prefectural University of Medicine, Japan.
Am J Gastroenterol. 1988 Feb;83(2):164-8.
A 20-yr-old woman with known diabetes insipidus complained of high fever and jaundice. Liver specimens biopsied under peritoneoscopy showed proliferating histiocytic or foamy cells in periportal areas. Immunocytochemical investigation demonstrated the presence of S-100 protein, but the absence of lysozyme in their histiocytic cells, and ultrastructural study revealed Birbeck granules in them. Vinblastine therapy reduced jaundice and high fever, and diabetes insipidus was controlled. Clinical, immunohistological, and cytological features suggested Letter-Siwe disease with jaundice, which responded well to treatment.
一名患有尿崩症的20岁女性出现高热和黄疸。经腹腔镜检查活检的肝脏标本显示，汇管区有组织细胞或泡沫细胞增生。免疫细胞化学研究表明，其组织细胞中存在S-100蛋白，但不存在溶菌酶，超微结构研究显示其中有伯贝克颗粒。长春花碱治疗减轻了黄疸和高热，尿崩症得到控制。临床、免疫组织学和细胞学特征提示为伴有黄疸的勒-雪病，对治疗反应良好。
