Association of monoclonal expansion of Epstein-Barr virus-negative CD158a+ NK cells secreting large amounts of gamma interferon with hemophagocytic lymphohistiocytosis.
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.
López-Alvarez
María R
MR
Inmunology Service, Hospital Universitario Virgen de Arrixaca, Murcia, Spain.
Martínez-Sánchez
María V
MV
Salgado-Cecilia
María G
MG
Campillo
José A
JA
Heine-Suñer
Damian
D
Villar-Permuy
Florentina
F
Fuster
José L
JL
Bas
Agueda
A
Gil-Herrera
Juana
J
Muro
Manuel
M
García-Alonso
Ana M
AM
Alvarez-López
María R
MR
Minguela
Alfredo
A
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*
D054342:Receptors, KIR2DL1; D007371:Interferon-gamma