title: Langerhans cell histiocytosis:  40 years' experience.
abstract: OBJECTIVE
  Our study analyzes 40 years' experience with pediatric Langerhans cell histiocytosis patients.
  
  
  METHODS
  Between June 1968 and December 2009, 121 patients (79 males, 42 females; median age 4.13 y) were diagnosed at our center (74% monosystemic disease; 26% multisystemic), treated according to current protocols. We evaluated the response, the survival, and the neuroendocrinological sequelae.
  
  
  RESULTS
  Overall survival (OS) for all patients was 93% at 10 years from diagnosis, event-free survival (EFS) 77%. OS for patients younger than 2 years and older than or equal to 2 years was 82% and 97% (P = 0.003); EFS 48% and 87% (P = 0.001). OS for patients diagnosed before and after April 1, 1991 was 84% and 98% (P = 0.007), EFS 66% and 85% (P = 0.03). OS for monosystemic and multisystemic disease was 100% and 71% (P < 0.001); EFS 88% and 45% (P < 0.001). OS for "risk" patients (involvement of bone marrow, spleen, liver, lungs) and "low-risk" patients was 50% and 94% (P = 0.007), EFS 37% and 54% (P = 0.06). Fourteen patients developed diabetes insipidus, 7 patients growth hormone deficiency, 2 hypothyroidism, and 1 neurodegeneration.
  
  
  CONCLUSIONS
  Our study confirms improvement of pathogenetic knowledge and treatment over the last 20 years. Age at diagnosis older than or equal to 2 years and standardized treatment are associated with improved prognoses. Multisystemic involvement, especially with "risk" organs seem to be correlated to a worse outcome.
authors.affiliation: Stem Cell Transplantation and Cellular Therapy Division, University of Turin, Turin, Italy. annapostini@hotmail.it
authors.forename: Anna
authors.initials: A
authors.lastname: Maria Postini
authors.forename: Adalberto Brach
authors.initials: AB
authors.lastname: del Prever
authors.forename: Manuela
authors.initials: M
authors.lastname: Pagano
authors.forename: Elisa
authors.initials: E
authors.lastname: Rivetti
authors.forename: Massimo
authors.initials: M
authors.lastname: Berger
authors.forename: Sebastian Dorin
authors.initials: SD
authors.lastname: Asaftei
authors.forename: Veronica
authors.initials: V
authors.lastname: Barat
authors.forename: Antonio
authors.initials: A
authors.lastname: Andreacchio
authors.forename: Franca
authors.initials: F
authors.lastname: Fagioli
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male