title: Impaired natural killer activity and expression of interleukin-2 receptor antigen in familial erythrophagocytic lymphohistiocytosis.
abstract: A 1-month-old boy with familial erythrophagocytic lymphohistiocytosis (FEL) had a barely detectable natural killer (NK) activity of 0% to 7% (median, 0.5%) with an effector/target ratio of 20:1. The number of Leu7+ and Leu11+ cells was within normal range. In terms of interleukin-2 (IL-2) receptor antigens, IL-2R/p55 (Tac) was marginally expressed whereas IL-2R/p75 related antigen recognized by YTA-1 monoclonal antibody (MAb),i.e., YTA-1 antigen, was moderately expressed on the patient's mononuclear cells. Since the NK activity was restored in vitro by IL-2 stimulation, insufficient in vivo IL-2 production or altered cooperation of IL-2R/p75 and IL-2R/p55 (Tac) in the IL-2 mediated immune response was suspected to be present. The induction of IL-2R/p55 (Tac) in vitro was found to be imparied after stimulation with IL-2, or YTA-1MAb. When the patient attained remission the IL-2R/p55 (Tac) induction had normalized, but low NK activity persisted. The results indicate that the IL-2/IL-2R system may play an important role in the etiology and pathogenesis of FEL.
authors.affiliation: Department of Pediatrics, Kyoto Prefectural University of Medicine, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Kataoka
authors.forename: S
authors.initials: S
authors.lastname: Todo
authors.forename: Y
authors.initials: Y
authors.lastname: Morioka
authors.forename: K
authors.initials: K
authors.lastname: Sugie
authors.forename: Y
authors.initials: Y
authors.lastname: Nakamura
authors.forename: J
authors.initials: J
authors.lastname: Yodoi
authors.forename: S
authors.initials: S
authors.lastname: Imashuku
mesh_terms: D000911:Antibodies, Monoclonal / Q000494:pharmacology; D002478:Cells, Cultured; D015616:Histiocytosis, Non-Langerhans-Cell / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007231:Infant, Newborn; D007376:Interleukin-2 / Q000494:pharmacology; D007694:Killer Cells, Natural / Q000187:drug effects / Q000276:immunology*; D008214:Lymphocytes / Q000187:drug effects / Q000276:immunology; D008297:Male; D009000:Monocytes / Q000187:drug effects / Q000276:immunology; D015375:Receptors, Interleukin-2 / Q000032:analysis*; D011994:Recombinant Proteins / Q000494:pharmacology; D012074:Remission Induction
chemical_list: D000911:Antibodies, Monoclonal; D007376:Interleukin-2; D015375:Receptors, Interleukin-2; D011994:Recombinant Proteins