[Deteriorating natural killer activity in familial erythrophagocytic lymphohistiocytosis].
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.
Caballero García
M
M
Centro Materno-Infantil, Hospital 1. de Octubre, Madrid.
Mar Molinero
F
F
Melián Machín
A
A
Sánchez Díaz
J I
JI
Mollejo Villanueva
M
M
Sotelo
M T
MT
Nogales Espert
A
A
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
Actividad "natural killer" deteriorada en la linfohistiocitosis eritrofagocítica familiar.