title: Impact of anakinra treatment on cytokine and lymphocytes/ monocytes profile of an Erdheim-Chester patient.
abstract: BACKGROUND
  Erdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis associated with intense immune activation. In our clinical center, an ECD patient was treated with anakinra, IL1RA (interleukin1 receptor antagonist), resulting in clinical improvement and major decrease of pathological fatigue. The aim of the study was to evaluate changes in cytokine profile and shift of immune cells estimated by flow cytometric analysis of ECD patient before, during initial stages of anakinra treatment as well as after treatment ceased in comparison to healthy donors.
  
  
  METHODS
  Singleplex reactions of 19 individual cytokines from serum of ECD patient were measured by FACS array. Flow cytometric analyses were performed on peripheral blood cells.
  
  
  RESULTS
  The most striking result is substantial decrease of IL6 immediately after anakinra treatment started suggesting a major role of IL1 pathway in ECD pathophysiology. As for flow cytometric analysis, increased number of CD16+ monocytes before treatment is a new finding.
  
  
  CONCLUSIONS
  Our results suggest that IL6 may be a marker of early treatment response of ECD patients treated with anakinra.
authors.forename: S
authors.initials: S
authors.lastname: Sevčíková
authors.forename: L
authors.initials: L
authors.lastname: Kubiczková
authors.forename: L
authors.initials: L
authors.lastname: Sedlaříková
authors.forename: L
authors.initials: L
authors.lastname: Ríhová
authors.forename: F
authors.initials: F
authors.lastname: Kryukov
authors.forename: P
authors.initials: P
authors.lastname: Szturz
authors.forename: R
authors.initials: R
authors.lastname: Hajek
authors.forename: L
authors.initials: L
authors.lastname: Pour
authors.forename: Z
authors.initials: Z
authors.lastname: Adam
mesh_terms: D016022:Case-Control Studies; D016207:Cytokines / Q000097:blood*; D031249:Erdheim-Chester Disease / Q000097:blood / Q000188:drug therapy*; D005434:Flow Cytometry; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008297:Male; D009000:Monocytes / Q000166:cytology*; D013601:T-Lymphocytes / Q000166:cytology*
chemical_list: D016207:Cytokines; D053590:Interleukin 1 Receptor Antagonist Protein