朗格汉斯细胞组织细胞增多症

Langerhans cell histiocytosis.

作者信息

Chauffaille M de L, Valério R M, Diniz C M, Simões M M, Enokihara S, Michalany N, Ferreira K V, Martinez J A, Hassun K M, Atallah A N, Kerbauy J

机构信息

Disciplina de Hematologia e Hemoterapia, Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil.

出版信息

Sao Paulo Med J. 1998 Jan-Feb;116(1):1625-8. doi: 10.1590/s1516-31801998000100006.

DOI:10.1590/s1516-31801998000100006

PMID:9699385

Abstract

The authors present a rare case of Langerhans cell histiocytosis in a 31 year old female patient with vulvar, peri-anal and oral lesions, diabetes insipidus, pulmonary skin and bone infiltrations. Skin biopsy immunohistochemistry presented positive S100 protein and vimentin, but the diagnosis was done with the demonstration of Birbeck granules with electronic microscopy. The treatment was based on systematical chemotherapy although vulvar lesion has a bad response to chemotherapy.

摘要

作者报告了一例罕见的朗格汉斯细胞组织细胞增多症，患者为一名31岁女性，有外阴、肛周和口腔病变，伴有尿崩症、肺部、皮肤和骨骼浸润。皮肤活检免疫组化显示S100蛋白和波形蛋白呈阳性，但诊断是通过电子显微镜下发现Birbeck颗粒来完成的。治疗基于系统化疗，尽管外阴病变对化疗反应不佳。