title: [Deteriorating natural killer activity in familial erythrophagocytic lymphohistiocytosis].
abstract: We report the occurrence of familial erythrophagocytic lymphohistiocytosis disease in a child who had greater involvement of the brain than of visceral organs. The diagnosis was made during life, allowing us to carry out biological, histological and immunological studies. The natural Killer activity was abolished in this patient. The combination chemotherapy--including systemic administration of VP 16-213, steroids and intrathecal methotrexate--, failed to stem the rapid course of the disease.
authors.affiliation: Centro Materno-Infantil, Hospital 1. de Octubre, Madrid.
authors.forename: M
authors.initials: M
authors.lastname: Caballero García
authors.forename: F
authors.initials: F
authors.lastname: Mar Molinero
authors.forename: A
authors.initials: A
authors.lastname: Melián Machín
authors.forename: J I
authors.initials: JI
authors.lastname: Sánchez Díaz
authors.forename: M
authors.initials: M
authors.lastname: Mollejo Villanueva
authors.forename: M T
authors.initials: MT
authors.lastname: Sotelo
authors.forename: A
authors.initials: A
authors.lastname: Nogales Espert
mesh_terms: D000917:Antibody Formation; D006801:Humans; D007111:Immunity, Cellular; D007223:Infant; D007694:Killer Cells, Natural / Q000503:physiopathology*; D008206:Lymphatic Diseases / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D008297:Male
vernacular_title: Actividad "natural killer" deteriorada en la linfohistiocitosis eritrofagocítica familiar.