Open AccessAssociation of Monoclonal Expansion of Epstein-Barr Virus-Negative CD158a + NK Cells Secreting Large Amounts of Gamma Interferon with Hemophagocytic Lymphohistiocytosis
Author(s) -
María R. López-Álvarez,
María V. MartínezSánchez,
María G. Salgado-Cecilia,
José A. Campillo,
Damián Heine-Suñer,
Florentina Villar-Permuy,
José Luís Fuster,
Águeda Bas,
Juana Gil-Herrera,
Manuel Muro,
Ana M. GarcíaAlonso,
María R. Álvarez-López,
Alfredo Minguela
Publication year - 2009
Publication title -
clinical and vaccine immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.649
H-Index - 77
eISSN - 1556-6811
pISSN - 1556-679X
DOI - 10.1128/cvi.00358-08
Subject(s) - hemophagocytic lymphohistiocytosis , epstein–barr virus , virus , virology , monoclonal antibody , immunology , interferon gamma , biology , medicine , antibody , cytokine , pathology , disease
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.