title: Serum levels of interleukin-1 receptor antagonist and tumor necrosis factor-alpha are elevated in children with Langerhans cell histiocytosis.
abstract: OBJECTIVE
  Langerhans cell histiocytosis (LCH) is a rare disease with variable prognosis in which lesions and clinical features suggest that pro- and anti-inflammatory cytokines may be involved in its pathogenesis. The authors wished to evaluate whether serum levels of interleukin-1 receptor agonist (IL-1Ra) and tumor necrosis factor-alpha (TNF-alpha) are elevated in children with LCH and decrease after chemotherapy.
  
  
  METHODS
  Circulating levels of IL-1Ra and TNF-alpha were measured in 23 and 8 children with LCH, respectively, and 7 pediatric controls using commercially available ELISA kits. All patients fulfilled the Histiocyte Society LCH Protocols criteria for diagnosis, stratification, and treatment.
  
  
  RESULTS
  Pretreatment concentrations of IL-1Ra and TNF-alpha were found to be significantly elevated in patients with LCH compared with controls. Among LCH substages, a significant difference in IL-1Ra values was observed between individuals with single-system single-site disease vs. multisystem disease with risk-organ dysfunction. In all eight patients evaluated, IL-1Ra levels decreased after 6 weeks of chemotherapy. Lower values of TNF were observed in three patients after treatment. A positive and significant correlation between IL-1Ra and TNF serum concentrations was found.
  
  
  CONCLUSIONS
  Patients with LCH have elevated levels of IL-1Ra and TNF, which decrease after chemotherapy.
authors.affiliation: Department of Hematology-Oncology, Hospital de Pediatría Dr. JP Garrahan, Buenos Aires, Argentina. drosso_2000@yahoo.com
authors.forename: Diego Alfredo
authors.initials: DA
authors.lastname: Rosso
authors.forename: Mario Francisco
authors.initials: MF
authors.lastname: Ripoli
authors.forename: Adriana
authors.initials: A
authors.lastname: Roy
authors.forename: Roberto A
authors.initials: RA
authors.lastname: Diez
authors.forename: Marta E
authors.initials: ME
authors.lastname: Zelazko
authors.forename: Jorge Luis
authors.initials: JL
authors.lastname: Braier
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000097:blood* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007223:Infant; D053590:Interleukin 1 Receptor Antagonist Protein; D008297:Male; D011237:Predictive Value of Tests; D011239:Prednisolone / Q000008:administration & dosage; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors; D011994:Recombinant Proteins; D012795:Sialoglycoproteins / Q000097:blood*; D014409:Tumor Necrosis Factor-alpha / Q000378:metabolism*; D014747:Vinblastine / Q000008:administration & dosage
chemical_list: C507763:IL1RN protein, human; D053590:Interleukin 1 Receptor Antagonist Protein; D017472:Receptors, Interleukin-1; D011994:Recombinant Proteins; D012795:Sialoglycoproteins; D014409:Tumor Necrosis Factor-alpha; D014747:Vinblastine; D005047:Etoposide; D011239:Prednisolone