title: Association of monoclonal expansion of Epstein-Barr virus-negative CD158a+ NK cells secreting large amounts of gamma interferon with hemophagocytic lymphohistiocytosis.
abstract: We report the first case of hemophagocytic lymphohistiocytosis (HLH) induced by the monoclonal expansion of Epstein-Barr virus (EBV)-negative NK cells. Consanguinity of the patient's parents made it necessary to discard familial HLH in the patient and her sister with identical HLA markers and demonstrate that no cause other than the expansion of NK cells, which secrete high levels of gamma interferon, was inducing HLH in this patient.
authors.affiliation: Inmunology Service, Hospital Universitario Virgen de Arrixaca, Murcia, Spain.
authors.forename: María R
authors.initials: MR
authors.lastname: López-Alvarez
authors.forename: María V
authors.initials: MV
authors.lastname: Martínez-Sánchez
authors.forename: María G
authors.initials: MG
authors.lastname: Salgado-Cecilia
authors.forename: José A
authors.initials: JA
authors.lastname: Campillo
authors.forename: Damian
authors.initials: D
authors.lastname: Heine-Suñer
authors.forename: Florentina
authors.initials: F
authors.lastname: Villar-Permuy
authors.forename: José L
authors.initials: JL
authors.lastname: Fuster
authors.forename: Agueda
authors.initials: A
authors.lastname: Bas
authors.forename: Juana
authors.initials: J
authors.lastname: Gil-Herrera
authors.forename: Manuel
authors.initials: M
authors.lastname: Muro
authors.forename: Ana M
authors.initials: AM
authors.lastname: García-Alonso
authors.forename: María R
authors.initials: MR
authors.lastname: Alvarez-López
authors.forename: Alfredo
authors.initials: A
authors.lastname: Minguela
mesh_terms: D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D007223:Infant; D007371:Interferon-gamma / Q000378:metabolism*; D007694:Killer Cells, Natural / Q000737:chemistry* / Q000276:immunology / Q000821:virology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000276:immunology / Q000473:pathology*; D010290:Parents; D054342:Receptors, KIR2DL1 / Q000032:analysis*
chemical_list: D054342:Receptors, KIR2DL1; D007371:Interferon-gamma