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

Authors
  • Le Louet, Solenne1
  • Barkaoui, Mohamed-Aziz1
  • Miron, Jean1
  • Galambrun, Claire2
  • Aladjidi, Nathalie3
  • Chastagner, Pascal4
  • Kebaili, Kamila5
  • Armari-Alla, Corinne6
  • Lambilliotte, Anne7
  • Lejeune, Julien8
  • Moshous, Despina9, 10
  • Della Valle, Valeria11
  • Sileo, Chiara11
  • Ducou Le Pointe, Hubert11
  • Chateil, Jean-François3
  • Renolleau, Sylvain9
  • Piloquet, Jean-Eudes11
  • Portefaix, Aurelie12
  • Epaud, Ralph13
  • Chiron, Raphaël14
  • And 6 more
  • 1 Trousseau Hospital, 26 avenue du Dr Netter, Paris, 75012, France , Paris (France)
  • 2 Hôpital de la Timone, Marseille, France , Marseille (France)
  • 3 Centre Hospitalo-Universitaire de Bordeaux, Bordeaux, France , Bordeaux (France)
  • 4 Brabois-Enfants Hospital, Centre Hospitalo-Universitaire de Nancy, Vandœuvre-lès-Nancy, France , Vandœuvre-lès-Nancy (France)
  • 5 Institut d’Hémato-Oncologie Pediatrique, Lyon, France , Lyon (France)
  • 6 Centre Hospitalo-Universitaire de Grenoble, La Tronche, France , La Tronche (France)
  • 7 Centre Hospitalo-Universitaire de Lille, Lille, France , Lille (France)
  • 8 Centre Hospitalo-Universitaire de Tours, Tours, France , Tours (France)
  • 9 Necker Hospital, Assistance Publique–Hôpitaux de Paris, Paris, France , Paris (France)
  • 10 Sorbonne University, Paris, France , Paris (France)
  • 11 Trousseau Hospital, Assistance Publique–Hôpitaux de Paris, Paris, France , Paris (France)
  • 12 Lyon Hospices Civils, Lyon, France , Lyon (France)
  • 13 CHIC, Créteil, France , Créteil (France)
  • 14 Arnaud de Villeneuve Hospital, Montpellier, France , Montpellier (France)
  • 15 Service de Pneumologie Centre de référence des histiocytoses Hôpital Saint Louis, Paris, France , Paris (France)
  • 16 Paris University, INSERM U976, Paris, France , Paris (France)
  • 17 Paris-Saclay University, Boulogne-Billancourt, France , Boulogne-Billancourt (France)
Type
Published Article
Journal
Orphanet Journal of Rare Diseases
Publisher
Springer (Biomed Central Ltd.)
Publication Date
Sep 09, 2020
Volume
15
Issue
1
Identifiers
DOI: 10.1186/s13023-020-01495-5
Source
Springer Nature
Keywords
License
Green

Abstract

BackgroundLung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed.MethodsAmong 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.ResultsThe 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.ConclusionsSevere 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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