Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
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(2020) 15:241
RESEARCH
Open Access
Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study Solenne Le Louet1* , Mohamed-Aziz Barkaoui1, Jean Miron1, Claire Galambrun2, Nathalie Aladjidi3, Pascal Chastagner4, Kamila Kebaili5, Corinne Armari-Alla6, Anne Lambilliotte7, Julien Lejeune8, Despina Moshous9,10, Valeria Della Valle11, Chiara Sileo11, Hubert Ducou Le Pointe11, Jean-François Chateil12, Sylvain Renolleau13, Jean-Eudes Piloquet14, Aurelie Portefaix15, Ralph Epaud16, Raphaël Chiron17, Emmanuelle Bugnet18, Gwenaël Lorillon18, Abdelatif Tazi18,19, Jean-François Emile20, Jean Donadieu1,21 and Sébastien Héritier1,20,21
Abstract 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. Keywords: Childhood, Pulmonary, Langerhans cell histiocytosis, Targeted therapy, Intensive care, Severe
* Correspondence: [email protected] 1 French Reference Center for Langerhans Cell Histiocytosis, Trousseau Hospital, 26 avenue du Dr Netter, 75012 Paris, France Full list of author information is available at the end of the article © The Author(s). 2020 Open Access This 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
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