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Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

BACKGROUND: Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed. METHODS: Among 1482 children (< 15 years) registered in the French LCH registry (1994–2018), 111 (7.4%) had lung involvemen...

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Autores principales: Le Louet, Solenne, Barkaoui, Mohamed-Aziz, Miron, Jean, Galambrun, Claire, Aladjidi, Nathalie, Chastagner, Pascal, Kebaili, Kamila, Armari-Alla, Corinne, Lambilliotte, Anne, Lejeune, Julien, Moshous, Despina, Della Valle, Valeria, Sileo, Chiara, Ducou Le Pointe, Hubert, Chateil, Jean-François, Renolleau, Sylvain, Piloquet, Jean-Eudes, Portefaix, Aurelie, Epaud, Ralph, Chiron, Raphaël, Bugnet, Emmanuelle, Lorillon, Gwenaël, Tazi, Abdelatif, Emile, Jean-François, Donadieu, Jean, Héritier, Sébastien
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7487928/
https://www.ncbi.nlm.nih.gov/pubmed/32907615
http://dx.doi.org/10.1186/s13023-020-01495-5
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author Le Louet, Solenne
Barkaoui, Mohamed-Aziz
Miron, Jean
Galambrun, Claire
Aladjidi, Nathalie
Chastagner, Pascal
Kebaili, Kamila
Armari-Alla, Corinne
Lambilliotte, Anne
Lejeune, Julien
Moshous, Despina
Della Valle, Valeria
Sileo, Chiara
Ducou Le Pointe, Hubert
Chateil, Jean-François
Renolleau, Sylvain
Piloquet, Jean-Eudes
Portefaix, Aurelie
Epaud, Ralph
Chiron, Raphaël
Bugnet, Emmanuelle
Lorillon, Gwenaël
Tazi, Abdelatif
Emile, Jean-François
Donadieu, Jean
Héritier, Sébastien
author_facet Le Louet, Solenne
Barkaoui, Mohamed-Aziz
Miron, Jean
Galambrun, Claire
Aladjidi, Nathalie
Chastagner, Pascal
Kebaili, Kamila
Armari-Alla, Corinne
Lambilliotte, Anne
Lejeune, Julien
Moshous, Despina
Della Valle, Valeria
Sileo, Chiara
Ducou Le Pointe, Hubert
Chateil, Jean-François
Renolleau, Sylvain
Piloquet, Jean-Eudes
Portefaix, Aurelie
Epaud, Ralph
Chiron, Raphaël
Bugnet, Emmanuelle
Lorillon, Gwenaël
Tazi, Abdelatif
Emile, Jean-François
Donadieu, Jean
Héritier, Sébastien
author_sort Le Louet, Solenne
collection PubMed
description BACKGROUND: Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed. METHODS: Among 1482 children (< 15 years) registered in the French LCH registry (1994–2018), 111 (7.4%) had lung involvement. This retrospective study included data for 17 (1.1%) patients that required one or more intensive care unit (ICU) admissions for respiratory failure. RESULTS: The median age was 1.3 years at the first ICU hospitalization. Of the 17 patients, 14 presented with lung involvement at the LCH diagnosis, and 7 patients (41%) had concomitant involvement of risk-organ (hematologic, spleen, or liver). Thirty-five ICU hospitalizations were analysed. Among these, 22 (63%) were secondary to a pneumothorax, 5 (14%) were associated with important cystic lesions without pneumothorax, and 8 (23%) included a diffuse micronodular lung infiltration in the context of multisystem disease. First-line vinblastine–corticosteroid combination therapy was administered to 16 patients; 12 patients required a second-line therapy (cladribine: n = 7; etoposide-aracytine: n = 3; targeted therapy n = 2). A total of 6 children (35%) died (repeated pneumothorax: n = 3; diffuse micronodular lung infiltration in the context of multisystem disease: n = 2; following lung transplantation: n = 1). For survivors, the median follow-up after ICU was 11.2 years. Among these, 9 patients remain asymptomatic despite abnormal chest imaging. CONCLUSIONS: Severe lung involvement is unusual in childhood LCH, but it is associated with high mortality. Treatment guidelines should be improved for this group of patients: viral infection prophylaxis and early administration of a new LCH therapy, such as targeted therapy.
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spelling pubmed-74879282020-09-16 Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study Le Louet, Solenne Barkaoui, Mohamed-Aziz Miron, Jean Galambrun, Claire Aladjidi, Nathalie Chastagner, Pascal Kebaili, Kamila Armari-Alla, Corinne Lambilliotte, Anne Lejeune, Julien Moshous, Despina Della Valle, Valeria Sileo, Chiara Ducou Le Pointe, Hubert Chateil, Jean-François Renolleau, Sylvain Piloquet, Jean-Eudes Portefaix, Aurelie Epaud, Ralph Chiron, Raphaël Bugnet, Emmanuelle Lorillon, Gwenaël Tazi, Abdelatif Emile, Jean-François Donadieu, Jean Héritier, Sébastien Orphanet J Rare Dis Research BACKGROUND: Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed. METHODS: Among 1482 children (< 15 years) registered in the French LCH registry (1994–2018), 111 (7.4%) had lung involvement. This retrospective study included data for 17 (1.1%) patients that required one or more intensive care unit (ICU) admissions for respiratory failure. RESULTS: The median age was 1.3 years at the first ICU hospitalization. Of the 17 patients, 14 presented with lung involvement at the LCH diagnosis, and 7 patients (41%) had concomitant involvement of risk-organ (hematologic, spleen, or liver). Thirty-five ICU hospitalizations were analysed. Among these, 22 (63%) were secondary to a pneumothorax, 5 (14%) were associated with important cystic lesions without pneumothorax, and 8 (23%) included a diffuse micronodular lung infiltration in the context of multisystem disease. First-line vinblastine–corticosteroid combination therapy was administered to 16 patients; 12 patients required a second-line therapy (cladribine: n = 7; etoposide-aracytine: n = 3; targeted therapy n = 2). A total of 6 children (35%) died (repeated pneumothorax: n = 3; diffuse micronodular lung infiltration in the context of multisystem disease: n = 2; following lung transplantation: n = 1). For survivors, the median follow-up after ICU was 11.2 years. Among these, 9 patients remain asymptomatic despite abnormal chest imaging. CONCLUSIONS: Severe lung involvement is unusual in childhood LCH, but it is associated with high mortality. Treatment guidelines should be improved for this group of patients: viral infection prophylaxis and early administration of a new LCH therapy, such as targeted therapy. BioMed Central 2020-09-09 /pmc/articles/PMC7487928/ /pubmed/32907615 http://dx.doi.org/10.1186/s13023-020-01495-5 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. 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 in a credit line to the data.
spellingShingle Research
Le Louet, Solenne
Barkaoui, Mohamed-Aziz
Miron, Jean
Galambrun, Claire
Aladjidi, Nathalie
Chastagner, Pascal
Kebaili, Kamila
Armari-Alla, Corinne
Lambilliotte, Anne
Lejeune, Julien
Moshous, Despina
Della Valle, Valeria
Sileo, Chiara
Ducou Le Pointe, Hubert
Chateil, Jean-François
Renolleau, Sylvain
Piloquet, Jean-Eudes
Portefaix, Aurelie
Epaud, Ralph
Chiron, Raphaël
Bugnet, Emmanuelle
Lorillon, Gwenaël
Tazi, Abdelatif
Emile, Jean-François
Donadieu, Jean
Héritier, Sébastien
Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
title Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
title_full Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
title_fullStr Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
title_full_unstemmed Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
title_short Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
title_sort childhood langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7487928/
https://www.ncbi.nlm.nih.gov/pubmed/32907615
http://dx.doi.org/10.1186/s13023-020-01495-5
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