title: Importance of multi-lineage hematologic involvement and hypoalbuminemia at diagnosis in patients with "risk-organ" multi-system Langerhans cell histiocytosis.
abstract: OBJECTIVE
  To perform a risk factor analysis in patients with "risk organ" multi-system Langerhans cell histiocytosis at diagnosis.
  
  
  METHODS
  From 1987 to 2007, 77 patients were analyzed. A univariate analysis of the variables, age <2 years, lungs, spleen and hepatic involvement, presence of >or=2 risk involved organs, hypoalbuminemia and the presence of isolated anemia, anemia with thrombocytopenia with or without leukopenia at diagnosis was performed. Statistically significant variables were combined and entered into a multivariate analysis.
  
  
  RESULTS
  Fifty-six and 66 evaluable patients had hematologic and hepatic involvement at diagnosis, respectively. Among the hematologic patients, the subgroup of anemia with thrombocytopenia with or without leukopenia showed a significantly lower 5-year survival than the subgroup of isolated anemia (0.19 vs. 0.87, respectively; P=0.0001). Of all the patients, those with hypoalbuminemia had a 5-year survival of 0.16 compared with those with normal albumin levels, who had a 5-year survival of 0.65 (P<0.0001). In multivariate analysis, only anemia with thrombocytopenia with or without leukopenia and hypoalbuminemia were the independent risk factors (relative risk 3.77; confidence interval, 1.7-8.4; P<0.0011 and relative risk 2.59; confidence interval, 1.24-5.4; P<0.0112).
  
  
  CONCLUSIONS
  Anemia with thrombocytopenia with or without leukopenia and hypoalbuminemia, were associated with worse prognosis in multi-system Langerhans cell histiocytosis. Other therapeutic strategies should be considered at diagnosis or early during the initial treatment for this high risk subgroup of patients.
authors.affiliation: Department of Hematology/Oncology, Hospital Nacional de Pediatría Juan P Garrahan, University of Buenos Aires, Buenos Aires, Argentina. jolubraier@gmail.com
authors.forename: Jorge Luis
authors.initials: JL
authors.lastname: Braier
authors.forename: Diego
authors.initials: D
authors.lastname: Rosso
authors.forename: Antonio
authors.initials: A
authors.lastname: Latella
authors.forename: Guillermo
authors.initials: G
authors.lastname: Chantada
authors.forename: Blanca
authors.initials: B
authors.lastname: Ozuna
authors.forename: Mario
authors.initials: M
authors.lastname: Ripoli
authors.forename: Marcelo
authors.initials: M
authors.lastname: Scopinaro
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000740:Anemia / Q000150:complications / Q000401:mortality / Q000473:pathology*; D019070:Cell Lineage; D002648:Child; D002675:Child, Preschool; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D006801:Humans; D034141:Hypoalbuminemia / Q000150:complications / Q000401:mortality / Q000473:pathology*; D007223:Infant; D007231:Infant, Newborn; D007970:Leukopenia / Q000150:complications / Q000401:mortality / Q000473:pathology*; D008297:Male; D012307:Risk Factors; D015996:Survival Rate; D013921:Thrombocytopenia / Q000150:complications / Q000401:mortality / Q000473:pathology*; D016896:Treatment Outcome