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Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report

BACKGROUND: Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects – by order of decreasing frequency – the bone, the skin, t...

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Autores principales: Mansour, Mohamad Jihad, Mokbel, Elias, Fares, Eddy, Maddah, Janah, Nasr, Fadi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5611558/
https://www.ncbi.nlm.nih.gov/pubmed/28942734
http://dx.doi.org/10.1186/s13256-017-1428-7
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author Mansour, Mohamad Jihad
Mokbel, Elias
Fares, Eddy
Maddah, Janah
Nasr, Fadi
author_facet Mansour, Mohamad Jihad
Mokbel, Elias
Fares, Eddy
Maddah, Janah
Nasr, Fadi
author_sort Mansour, Mohamad Jihad
collection PubMed
description BACKGROUND: Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects – by order of decreasing frequency – the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adults. CASE PRESENTATION: We describe the case of a 32-year-old Middle Eastern man with Langerhans cell histiocytosis involving his lungs and the colorectoanal part of his gastrointestinal tract, with complete resolution of gastrointestinal tract lesions following a non-standardized chemotherapy regimen. CONCLUSIONS: Gastrointestinal tract lesions are a rare manifestation of Langerhans cell histiocytosis, especially when associated with extraintestinal involvement, such as the lungs. Chemotherapy protocols have not been well established for the treatment of the disease. The clinical impact of the effective chemotherapy regimen used to treat this uncommon presentation of Langerhans cell histiocytosis will be viewed in this case report. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s13256-017-1428-7) contains supplementary material, which is available to authorized users.
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spelling pubmed-56115582017-10-11 Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report Mansour, Mohamad Jihad Mokbel, Elias Fares, Eddy Maddah, Janah Nasr, Fadi J Med Case Rep Case Report BACKGROUND: Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects – by order of decreasing frequency – the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adults. CASE PRESENTATION: We describe the case of a 32-year-old Middle Eastern man with Langerhans cell histiocytosis involving his lungs and the colorectoanal part of his gastrointestinal tract, with complete resolution of gastrointestinal tract lesions following a non-standardized chemotherapy regimen. CONCLUSIONS: Gastrointestinal tract lesions are a rare manifestation of Langerhans cell histiocytosis, especially when associated with extraintestinal involvement, such as the lungs. Chemotherapy protocols have not been well established for the treatment of the disease. The clinical impact of the effective chemotherapy regimen used to treat this uncommon presentation of Langerhans cell histiocytosis will be viewed in this case report. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s13256-017-1428-7) contains supplementary material, which is available to authorized users. BioMed Central 2017-09-25 /pmc/articles/PMC5611558/ /pubmed/28942734 http://dx.doi.org/10.1186/s13256-017-1428-7 Text en © The Author(s). 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Mansour, Mohamad Jihad
Mokbel, Elias
Fares, Eddy
Maddah, Janah
Nasr, Fadi
Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report
title Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report
title_full Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report
title_fullStr Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report
title_full_unstemmed Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report
title_short Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report
title_sort adult langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5611558/
https://www.ncbi.nlm.nih.gov/pubmed/28942734
http://dx.doi.org/10.1186/s13256-017-1428-7
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