title: CD14(dim)/CD16(bright) monocytes in hemophagocytic lymphohistiocytosis.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is an extremely rare and highly lethal chronic inflammatory disease, which is mediated by proinflammatory cytokines. In the peripheral blood of a boy suffering from HLH, a chronic expansion of CD14(dim)/CD16(bright) inflammatory monocytes was detected. Compared with CD14(bright) monocytes, their immunophenotype correlated with more mature monocytic cells differentiating to macrophages: they showed lower expression of CD11b, CD64 and CD35. Such CD14(dim)/CD16(bright) monocytes produce the inflammatory cytokines IL-1beta, IL-6 and TNF-alpha. They fit in well with the pathophysiological concept of HLH as an inflammatory state of lymphocytes and of the monocyte/macrophage system. In the presented patient the percentage of these circulating inflammatory monocytes decreased over time during clinical response to immunosuppressive therapy. This finding may indicate that CD14(dim)/CD16(bright) monocytes represented the degree of inflammation in this extremely rare and highly lethal disease.
authors.affiliation: University Children's Hospital, Vienna, Austria. wolfgang.emminger@kin.akh.magwien.gv.at
authors.forename: W
authors.initials: W
authors.lastname: Emminger
authors.forename: G J
authors.initials: GJ
authors.lastname: Zlabinger
authors.forename: G
authors.initials: G
authors.lastname: Fritsch
authors.forename: R
authors.initials: R
authors.lastname: Urbanek
mesh_terms: D000293:Adolescent; D002452:Cell Count; D016572:Cyclosporine / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D006684:HLA-DR Antigens / Q000096:biosynthesis; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D018950:Lipopolysaccharide Receptors / Q000276:immunology*; D016177:Macrophage-1 Antigen / Q000096:biosynthesis; D008297:Male; D009000:Monocytes / Q000166:cytology / Q000276:immunology*; D017463:Receptors, Complement 3b / Q000096:biosynthesis; D017452:Receptors, IgG / Q000096:biosynthesis / Q000276:immunology*
chemical_list: D006684:HLA-DR Antigens; D007166:Immunosuppressive Agents; D018950:Lipopolysaccharide Receptors; D016177:Macrophage-1 Antigen; D017463:Receptors, Complement 3b; D017452:Receptors, IgG; D005047:Etoposide; D003907:Dexamethasone; D016572:Cyclosporine