title: Increased cytokine levels and abnormal response of myeloid progenitor cells to granulocyte colony-stimulating factor in a case of severe congenital neutropenia. In vitro effects of stem cell factor.
abstract: OBJECTIVE
  The cytokine levels and the in vitro granulopoiesis were studied to evaluate the mechanism of impaired granulopoiesis in severe congenital neutropenia (SCN).
  
  
  METHODS
  The patient was a 5-year-old boy with SCN. We assayed the colony-stimulating activity (CSA) produced by peripheral blood (PB) cells from the patient. The plasma levels of cytokines were measured using enzyme immunoassay. These included granulocyte colony-stimulating factor (G-CSF), granulocyte-macrophage colony-stimulating factor (GM-CSF), interleukin 1 alpha (IL-1 alpha), IL-1 beta, IL-2, IL-3, IL-4, IL-6, and tumor necrosis factor-alpha. The effects of IL-3 and stem cell factor (SCF) on the proliferation of granulocyte-macrophage colony-forming cells (GM-CFCs) were studied.
  
  
  RESULTS
  CSA produced by PB cells from the patient was almost the same as in the healthy control. The level of endogenous G-CSF was elevated to 334 pg/ml, and GM-CSF, IL-2, IL-3, and IL-6 were slightly elevated. The numbers of GM-CFCs were markedly depressed in the presence of G-CSF alone and showed no increment on additional stimulation by IL-3. SCF in combination with G-CSF significantly augmented the proliferation of GM-CFCs.
  
  
  CONCLUSIONS
  These findings suggest that some cytokines including G-CSF may be elevated in SCN patients and that CSF may play an important role in the pathogenesis of SCN.
authors.affiliation: Department of Pediatrics, Gunma University School of Medicine, Maebashi City, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Shitara
authors.forename: H
authors.initials: H
authors.lastname: Ijima
authors.forename: S
authors.initials: S
authors.lastname: Yugami
authors.forename: M
authors.initials: M
authors.lastname: Sotomatu
authors.forename: T
authors.initials: T
authors.lastname: Kuroume
mesh_terms: D003115:Colony-Stimulating Factors / Q000096:biosynthesis; D016207:Cytokines / Q000097:blood*; D016179:Granulocyte Colony-Stimulating Factor / Q000494:pharmacology*; D016298:Hematopoietic Cell Growth Factors / Q000494:pharmacology*; D006412:Hematopoietic Stem Cells / Q000187:drug effects*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009503:Neutropenia / Q000151:congenital* / Q000209:etiology; D011994:Recombinant Proteins / Q000494:pharmacology; D019089:Stem Cell Factor
chemical_list: D003115:Colony-Stimulating Factors; D016207:Cytokines; D016298:Hematopoietic Cell Growth Factors; D011994:Recombinant Proteins; D019089:Stem Cell Factor; D016179:Granulocyte Colony-Stimulating Factor