title: Depressed levels of interferon-gamma and HLA-DR+CD3+ T cells in infants with transient hyperferritinemia.
abstract: Familial hemophagocytic lymphohistiocytosis (FHL), which typically has its onset during infancy, is uniformly fatal if not treated. It therefore requires prompt therapeutic intervention. Although hyperferritinemia has been emphasized as a useful marker for FHL, some nonfatal cases in infants with spontaneous remission also manifest with hyperferritinemia. However, distinguishing them is difficult because initial clinical features of these infants are similar. The authors encountered 14 infants with hyperferritinemia (serum ferritin >674 ng/mL), which normalized within 3 weeks following a benign clinical course. The authors compared the levels of HLA-DR+CD3+ T-cell subsets and interferon-gamma (IFN-γ) in the peripheral blood between these infants and FHL cases: one of the authors' own patients and others from the literature. Serum IFN-γ was not detected in infants with hyperferritinemia. Moreover, levels of HLA-DR+CD3+ T cells were extremely depressed. In contrast, serum IFN-γ was elevated and HLA-DR+CD3+ T cells were not depressed in FHL. Measurement of activated T cells and serum IFN-γ might help differentiate FHL in febrile infants with transient hyperferritinemia.
authors.affiliation: Department of Pediatrics, Tazuke Kofukai Medical Research Institute, Osaka, Japan. mi-shiota@kitano-hp.or.jp
authors.forename: Mitsutaka
authors.initials: M
authors.lastname: Shiota
authors.forename: Akira
authors.initials: A
authors.lastname: Kumakura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mizumoto
authors.forename: Junko
authors.initials: J
authors.lastname: Asada
authors.forename: Kenshi
authors.initials: K
authors.lastname: Nakagawa
authors.forename: Mayuko
authors.initials: M
authors.lastname: Takuwa
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Morishima
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Nishida
authors.forename: Takakazu
authors.initials: T
authors.lastname: Yoshioka
authors.forename: Atsuko
authors.initials: A
authors.lastname: Hata
authors.forename: Daisuke
authors.initials: D
authors.lastname: Hata
mesh_terms: D000293:Adolescent; D017252:CD3 Complex / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005293:Ferritins / Q000097:blood*; D006684:HLA-DR Antigens / Q000097:blood*; D006801:Humans; D007371:Interferon-gamma / Q000097:blood*; D019190:Iron Overload / Q000097:blood*; D008297:Male; D011379:Prognosis; D013601:T-Lymphocytes / Q000378:metabolism*
chemical_list: D017252:CD3 Complex; D006684:HLA-DR Antigens; D007371:Interferon-gamma; D005293:Ferritins