Cargando…
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...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , , , , |
---|---|
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 |
_version_ | 1783581589622489088 |
---|---|
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. |
format | Online Article Text |
id | pubmed-7487928 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT lelouetsolenne childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT barkaouimohamedaziz childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT mironjean childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT galambrunclaire childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT aladjidinathalie childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT chastagnerpascal childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT kebailikamila childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT armariallacorinne childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT lambilliotteanne childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT lejeunejulien childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT moshousdespina childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT dellavallevaleria childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT sileochiara childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT ducoulepointehubert childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT chateiljeanfrancois childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT renolleausylvain childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT piloquetjeaneudes childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT portefaixaurelie childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT epaudralph childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT chironraphael childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT bugnetemmanuelle childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT lorillongwenael childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT taziabdelatif childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT emilejeanfrancois childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT donadieujean childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy AT heritiersebastien childhoodlangerhanscellhistiocytosiswithseverelunginvolvementanationwidecohortstudy |