title: A new clinical score for disease activity in Langerhans cell histiocytosis.
abstract: OBJECTIVE
  To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH).
  
  
  METHODS
  Scoring system was developed and applied to a database containing information on 612 patients.
  
  
  RESULTS
  At diagnosis, the score distribution was highly asymmetrical: the score was between 0 and 2 in 74% of cases, 3-6 in 16%, 7-10 in 3%, and more than 10 in 6%. The 5-year mortality rates were 1, 4.4, and 43.4%, respectively, among patients with initial scores of 0-2, 3-6, and >6. Stability or an increase of the score at 6 weeks was highly predictive of death among patients with initial scores above 6, while score stability had no significant impact on vital outcome among patients with low or moderate scores at diagnosis.
  
  
  CONCLUSIONS
  This LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow-up and treatment.
authors.affiliation: Hôpital Trousseau, 26 avenue du Dr Netter, Paris, France. jean.donadieu@trs.ap-hop-paris.fr
authors.forename: Jean
authors.initials: J
authors.lastname: Donadieu
authors.forename: C
authors.initials: C
authors.lastname: Piguet
authors.forename: F
authors.initials: F
authors.lastname: Bernard
authors.forename: M
authors.initials: M
authors.lastname: Barkaoui
authors.forename: M
authors.initials: M
authors.lastname: Ouache
authors.forename: Y
authors.initials: Y
authors.lastname: Bertrand
authors.forename: H
authors.initials: H
authors.lastname: Ibrahim
authors.forename: J F
authors.initials: JF
authors.lastname: Emile
authors.forename: O
authors.initials: O
authors.lastname: Hermine
authors.forename: A
authors.initials: A
authors.lastname: Tazi
authors.forename: T
authors.initials: T
authors.lastname: Genereau
authors.forename: C
authors.initials: C
authors.lastname: Thomas
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003627:Data Interpretation, Statistical; D016208:Databases, Factual; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D006801:Humans; D011237:Predictive Value of Tests; D012720:Severity of Illness Index; D016008:Statistical Distributions; D016019:Survival Analysis; D013997:Time Factors