Korean J Gastrointest Endosc > Volume 26(1); 2003 > Article
Korean Journal of Gastrointestinal Endoscopy 2003;26(1): 56-60.
경화성 담관염을 동반한 랑게르한스 세포 조직구증 1예
아주대학교 의과대학 소화기내과학교실
A Case of Langerhans Cell Histiocytosis with Sclerosing Cholangitis
Jae Chul Hwang, M.D., Doh Hyun Kim, M.D., Eun Hee Lee, M.D., Jai Hak Jeung, M.D.,
Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea
Langerhans cell histiocytosis is a rare disorder with abnormal proliferation of histiocytes. Besides the infiltration of a variety of organs, patients with Langerhans cell histiocytosis can develop sclerosing cholangitis, with bile duct involvement, progressive fibrosis, and cirrhosis. We report a case of Langerhans cell histiocytosis with sclerosing cholangitis. Endoscopic retrograde cholangiopancreatography revealed multiple strictures and dilatations of left intrahepatic and extrahepatic bile duct. Endoscopic biopsy of common bile duct showed diffuse infiltration of histiocytes and eosinophiles in lamina propria. Immunohistochemical stain showed positive reactions for S-100 and CD1a and a negative reaction for cytokeratin in infiltrating histiocytes. (Korean J Gastrointest Endosc 2003;26:56⁣60)
Key Words: Langerhans cell histiocytosis, Sclerosing cholangitis, CD1a, S100
주요어: 랑게르한스세포, 조직구증, 경화성 담관염, CD1a, S100
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