Haas S, Theuerkauf I, Kühnen A, Wickesberg A, Fischer H-P
Institut für Pathologie, Rheinische Friedrich-Wilhelms-Universität Bonn,
Pathologe. 2003 Mar;24(2):119-23. doi: 10.1007/s00292-002-0565-x. Epub 2002 Nov 6.
We report on the difficult differential diagnosis of liver involvement in disseminated Langerhans' cell histiocytosis (LCH). Three years after treatment of LCH involving the skull and pelvic bones, an 18-year-old girl presented with abdominal pain and cholestatic liver disease. At this time, liver biopsy showed portal infiltrates which were diagnosed as chronic non-suppurative destructive cholangitis. Two years later, she was icteric under progredient hepatic failure. A second liver biopsy revealed biliary fibrosis and granulomatous inflammation with destruction of the portal bile ducts. The morphological changes in both liver biopsies could be identified as LCH by immunohistochemical detection of CD1a and S-100-positive Langerhans' cells. Morphological changes and clinical findings in LCH of the liver may resemble primary sclerosing cholangitis or chronic non-suppurative destructive cholangitis. Therefore, LCH is an important differential diagnosis of chronic destructive cholangitis with cholestatic liver disease, especially in children and young adults. The diagnosis can be verified by S-100 and CD1a immunohistochemistry.
我们报告了播散性朗格汉斯细胞组织细胞增多症（LCH）肝脏受累的困难鉴别诊断。一名18岁女孩在治疗累及颅骨和骨盆骨的LCH三年后，出现腹痛和胆汁淤积性肝病。此时，肝脏活检显示门脉浸润，诊断为慢性非化脓性破坏性胆管炎。两年后，她因进行性肝功能衰竭出现黄疸。第二次肝脏活检显示胆管纤维化和肉芽肿性炎症，伴有门脉胆管破坏。通过免疫组化检测CD1a和S-100阳性朗格汉斯细胞，可将两次肝脏活检中的形态学改变均鉴定为LCH。肝脏LCH的形态学改变和临床表现可能类似于原发性硬化性胆管炎或慢性非化脓性破坏性胆管炎。因此，LCH是胆汁淤积性肝病伴慢性破坏性胆管炎的重要鉴别诊断，尤其是在儿童和年轻人中。可通过S-100和CD1a免疫组化来证实诊断。
