Multisystem Langerhans cell histiocytosis with liver dysfunction as the first presentation: A case report

Liu,Dian-Gang; Zhang,Yu-Xian; Li,Fei

doi:10.3892/ol.2011.462

February 2012 Volume 3 Issue 2

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February 2012 Volume 3 Issue 2

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Case Report

Multisystem Langerhans cell histiocytosis with liver dysfunction as the first presentation: A case report

Authors:
- Dian-Gang Liu
- Yu-Xian Zhang
- Fei Li
View Affiliations / Copyright

Affiliations: Department of General Surgery, Xuanwu Hospital, Capital Medical University, Xuanwu, Beijing 100053, P.R. China, Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100053, P.R. China
Pages: 391-394
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Published online on: October 26, 2011

https://doi.org/10.3892/ol.2011.462
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Abstract

Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology characterized by oligoclonal proliferation of Langerhans cells. The diagnosis of LCH is complicated by the fact that it may involve multiple organ systems and its clinical presentation and course varies, ranging from an isolated to a multisystem disease. We report a 35-year‑old male with LCH involving multiple systems, including the bones, lungs, spleen, liver and bile ducts, whose first clinical presentation was liver dysfunction. The patient was diagnosed following a skull biopsy that revealed infiltration of Langerhans cells. However, a liver biopsy revealed sclerosing cholangitis (SC) with no signs of Langerhans cell infiltration, and the clinical manifestations of the involved organs were atypical, leading to a delayed diagnosis. The patient was in partial remission following chemotherapy. In conclusion, findings of this case may aid our understanding of the pathophysiology of LCH and in improving its diagnosis and treatment.

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Journals

International Journal of Molecular Medicine

International Journal of Oncology

Molecular Medicine Reports

Oncology Reports

Experimental and Therapeutic Medicine

Oncology Letters

Biomedical Reports

Molecular and Clinical Oncology

World Academy of Sciences Journal

International Journal of Functional Nutrition

International Journal of Epigenetics

Medicine International

Multisystem Langerhans cell histiocytosis with liver dysfunction as the first presentation: A case report

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Abstract

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