Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Altho...

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Main Authors: Bruno Araujo, Francisco Costa, Joanne Lopes, Ricardo Castro
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Radiology
Online Access:http://dx.doi.org/10.1155/2015/536328
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author Bruno Araujo
Francisco Costa
Joanne Lopes
Ricardo Castro
author_facet Bruno Araujo
Francisco Costa
Joanne Lopes
Ricardo Castro
author_sort Bruno Araujo
collection DOAJ
description Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.
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institution Kabale University
issn 2090-6862
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language English
publishDate 2015-01-01
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series Case Reports in Radiology
spelling doaj-art-b27a7d7b83d340cc926fa932bec8a35d2025-08-20T03:39:25ZengWileyCase Reports in Radiology2090-68622090-68702015-01-01201510.1155/2015/536328536328Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary InvolvementBruno Araujo0Francisco Costa1Joanne Lopes2Ricardo Castro3Radiology Department, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200–319 Porto, PortugalRadiology Department, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200–319 Porto, PortugalPathology Department, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200–319 Porto, PortugalRadiology Department, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200–319 Porto, PortugalLangerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.http://dx.doi.org/10.1155/2015/536328
spellingShingle Bruno Araujo
Francisco Costa
Joanne Lopes
Ricardo Castro
Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement
Case Reports in Radiology
title Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement
title_full Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement
title_fullStr Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement
title_full_unstemmed Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement
title_short Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement
title_sort adult langerhans cell histiocytosis with hepatic and pulmonary involvement
url http://dx.doi.org/10.1155/2015/536328
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AT franciscocosta adultlangerhanscellhistiocytosiswithhepaticandpulmonaryinvolvement
AT joannelopes adultlangerhanscellhistiocytosiswithhepaticandpulmonaryinvolvement
AT ricardocastro adultlangerhanscellhistiocytosiswithhepaticandpulmonaryinvolvement