title: [Langerhans cell histiocytosis in adults].
abstract: Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells, most often organized in granulomas. The disease has been initially described in children. The clinical picture of LCH is highly variable. Bone, skin, pituitary gland, lung, central nervous system, lymphoid organs are the main organs involved whereas liver and intestinal tract localizations are less frequently encountered. LCH course ranges from a fulminant multisystem disease to spontaneous resolution. Several randomized controlled trials have enable pediatricians to refine the management of children with LCH. Adult LCH has some specific features and poses distinct therapeutic challenges, knowing that data on these patients are limited. Herein, we will provide an overview of current knowledge regarding adult LCH and its management. We will also discuss recent advances in the understanding of the disease, (i.e. the role of BRAF oncogene) that opens the way toward targeted therapies.
authors.affiliation: Service de médecine interne, Hôtel-Dieu, CHU de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France. Electronic address: antoine.neel@univ-nantes.fr.
authors.forename: A
authors.initials: A
authors.lastname: Néel
authors.affiliation: Service de médecine interne, Hôtel-Dieu, CHU de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.
authors.forename: M
authors.initials: M
authors.lastname: Artifoni
authors.affiliation: Service d'hémato-oncologie pédiatrique, centre national de référence de l'histiocytose langerhansienne, hôpital Trousseau, AP-HP, 75012 Paris, France.
authors.forename: J
authors.initials: J
authors.lastname: Donadieu
authors.affiliation: Service de pneumologie, centre national de référence de l'histiocytose langerhansienne, hôpital Saint-Louis, AP-HP, 75475 Paris, France.
authors.forename: G
authors.initials: G
authors.lastname: Lorillon
authors.affiliation: Service de médecine interne, Hôtel-Dieu, CHU de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.
authors.forename: M
authors.initials: M
authors.lastname: Hamidou
authors.affiliation: Service de pneumologie, centre national de référence de l'histiocytose langerhansienne, hôpital Saint-Louis, AP-HP, 75475 Paris, France; Inserm UMR 717, université Paris Diderot, Sorbonne Paris Cité, 75012 Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Tazi
mesh_terms: D000328:Adult; D017668:Age of Onset; D003937:Diagnosis, Differential; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D006801:Humans; D009369:Neoplasms / Q000453:epidemiology / Q000209:etiology
keywords: Adult; Adulte; Cellule dendritique; Dendritic cells; Histiocytose langerhansienne; Langerhans cell histiocytosis; Traitement; Treatment