Liver and intrahepatic bile ducts - tumor
Other malignancies
Langerhans cell histiocytosis (LCH)

Topic Completed: 1 February 2012

Revised: 28 January 2019, last major update February 2012

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Langerhans cell histiocytosis liver[TI]

See also: Lymph nodes - not lymphoma chapter

Deepali Jain, M.D.
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Cite this page: Jain D. Langerhans cell histiocytosis (LCH). website. Accessed April 1st, 2020.
Definition / general
  • Neoplastic disorder of Langerhans cells, formerly called histiocytosis X
  • Causes 15% of sclerosing cholangitis in children, usually affects intrahepatic bile ducts
  • Remarkable selectivity for bile ducts, eventually causes biliary cirrhosis
  • May only affect liver; also affects lymph nodes, skin
  • Gamma glutamyl transpeptidase is sensitive indicator of liver infiltration in children with LCH
Case reports
  • 65 year old woman with sclerosing cholangitis 4 years after diagnosis of Langerhans cell histiocytosis (Hepatol Int 2010;4:653)
Microscopic (histologic) description
  • Langerhans cells may surround bile ducts and infiltrate sinusoids in nodules or diffusely
  • Associated with cirrhosis, sclerosing cholangitis, eosinophils, lymphocytes, plasma cells and neutrophils (Mod Pathol 1999;12:370)
Microscopic (histologic) images

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Langerhans cell histiocytosis - site unspecified

Sclerosing cholangitis caused by LCH but no LCH involvement of liver itself

Positive stains
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