title: Langerhans cell histiocytosis: differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre.
abstract: OBJECTIVE
  This study determined the frequency of clinical features, reactivations, sequelae, mortality, and overall survival (OS) and compared paediatric with adult Langerhans cell histiocytosis (LCH) patients.
  
  
  METHODS
  Ninety patients (60 paediatric and 30 adults) with LCH treated during 28 years were analysed retrospectively.
  
  
  RESULTS
  Craniofacial lesion was the most frequent lesion at LCH presentation in children and adults. However, some differences were found. Orbital lesions were more frequent in paediatric than adult patients (P = 0.001). There was a tendency for mandible lesions to be more common in adults than the paediatric group (P = 0.0710). Mucocutaneous lesions were observed in a higher proportion in adults compared to paediatric patients (P = 0.0395). Reactivation episodes (36.8 versus 62.5%) and deaths (10.7 versus 24.0%) occurred in lower proportions in paediatric than adult patients, respectively. The probability of OS in 10 years for both groups was similar (P = 0.137).
  
  
  CONCLUSIONS
  The OS was similar in both groups despite clinical differences between paediatric and adult patients, and higher reactivation and death rates in adults.
authors.forename: Raquel Ciuvalschi
authors.initials: RC
authors.lastname: Maia
authors.forename: Lídia Maria Magalhães
authors.initials: LM
authors.lastname: de Rezende
authors.forename: Marcela
authors.initials: M
authors.lastname: Robaina
authors.forename: Alexandre
authors.initials: A
authors.lastname: Apa
authors.forename: Claudete Esteves
authors.initials: CE
authors.lastname: Klumb
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005147:Facial Bones / Q000187:drug effects / Q000473:pathology / Q000601:surgery; D005260:Female; D005720:Gamma Rays / Q000627:therapeutic use*; D006646:Histiocytosis, Langerhans-Cell / Q000401:mortality / Q000473:pathology / Q000601:surgery* / Q000628:therapy*; D006801:Humans; D007223:Infant; D008168:Lung / Q000187:drug effects / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009092:Mucous Membrane / Q000187:drug effects / Q000473:pathology / Q000601:surgery; D011241:Prednisone / Q000627:therapeutic use*; D012189:Retrospective Studies; D016019:Survival Analysis
chemical_list: D000893:Anti-Inflammatory Agents; D011241:Prednisone
keywords: Adults; Children; Diabetes insipidus; Langerhans cells histiocytosis; Lytic bone lesions