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Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis

Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse cystic lung disease that occurs almost exclusively in young adult smokers. High-resolution computed tomography of the chest allows a confident diagnosis of PLCH in typical presentation, when nodules, cavitating nodules, and cysts...

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Autores principales: Fira-Mladinescu, Ovidiu, Suppini, Noemi, Olteanu, Gheorghe-Emilian, Fira-Mladinescu, Corneluta, Traila, Daniel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9221967/
https://www.ncbi.nlm.nih.gov/pubmed/35741204
http://dx.doi.org/10.3390/diagnostics12061394
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author Fira-Mladinescu, Ovidiu
Suppini, Noemi
Olteanu, Gheorghe-Emilian
Fira-Mladinescu, Corneluta
Traila, Daniel
author_facet Fira-Mladinescu, Ovidiu
Suppini, Noemi
Olteanu, Gheorghe-Emilian
Fira-Mladinescu, Corneluta
Traila, Daniel
author_sort Fira-Mladinescu, Ovidiu
collection PubMed
description Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse cystic lung disease that occurs almost exclusively in young adult smokers. High-resolution computed tomography of the chest allows a confident diagnosis of PLCH in typical presentation, when nodules, cavitating nodules, and cysts coexist and show a predominance for the upper and middle lung. Atypical presentations require histology for diagnosis. Histologic diagnosis rests on the demonstration of increased numbers of Langerhans cells and/or specific histological changes. PLCH is one of the few diseases in which bronchoalveolar lavage (BAL) has a high diagnostic value and can in some circumstances replace lung biopsy. We present a case of PLCH in an elderly non-smoker. Chest imaging revealed the presence of advanced interstitial lung disease with a fibrocystic pattern. BAL cellular analyses disclosed a macrophage pattern with CD1a phenotype that strongly supports the PLCH diagnosis, even in the setting of atypical clinical presentation and a lack of smoking exposure. PLCH is extremely rare in non-smokers and could represent a distinct phenotype.
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spelling pubmed-92219672022-06-24 Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis Fira-Mladinescu, Ovidiu Suppini, Noemi Olteanu, Gheorghe-Emilian Fira-Mladinescu, Corneluta Traila, Daniel Diagnostics (Basel) Interesting Images Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse cystic lung disease that occurs almost exclusively in young adult smokers. High-resolution computed tomography of the chest allows a confident diagnosis of PLCH in typical presentation, when nodules, cavitating nodules, and cysts coexist and show a predominance for the upper and middle lung. Atypical presentations require histology for diagnosis. Histologic diagnosis rests on the demonstration of increased numbers of Langerhans cells and/or specific histological changes. PLCH is one of the few diseases in which bronchoalveolar lavage (BAL) has a high diagnostic value and can in some circumstances replace lung biopsy. We present a case of PLCH in an elderly non-smoker. Chest imaging revealed the presence of advanced interstitial lung disease with a fibrocystic pattern. BAL cellular analyses disclosed a macrophage pattern with CD1a phenotype that strongly supports the PLCH diagnosis, even in the setting of atypical clinical presentation and a lack of smoking exposure. PLCH is extremely rare in non-smokers and could represent a distinct phenotype. MDPI 2022-06-04 /pmc/articles/PMC9221967/ /pubmed/35741204 http://dx.doi.org/10.3390/diagnostics12061394 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Interesting Images
Fira-Mladinescu, Ovidiu
Suppini, Noemi
Olteanu, Gheorghe-Emilian
Fira-Mladinescu, Corneluta
Traila, Daniel
Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis
title Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis
title_full Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis
title_fullStr Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis
title_full_unstemmed Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis
title_short Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis
title_sort bronchoalveolar lavage as a diagnostic tool in an atypical pulmonary langerhans cell histiocytosis
topic Interesting Images
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9221967/
https://www.ncbi.nlm.nih.gov/pubmed/35741204
http://dx.doi.org/10.3390/diagnostics12061394
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