CVI Accepts, published online ahead of print on 19 November 2008
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Clin. Vaccine Immunol. doi:10.1128/CVI.00358-08
Copyright (c) 2008, American Society for Microbiology and/or the Listed Authors/Institutions. All Rights Reserved.

Monoclonal expansion of EBV-negative CD158a+ NK cells secreting high amounts of IFN-{gamma} associated with hemophagocytic lymphohistiocytosis

María R. López-Álvarez, María V. Martínez-Sánchez, María G. Salgado-Cecilia, José A. Campillo, Damian Heine-Suñer, Florentina Villar-Permuy, José L. Fuster, Águeda Bas, Juana Gil-Herrera, Manuel Muro, Ana M. García-Alonso, María R. Álvarez-López, and Alfredo Minguela*

Inmunology, Pediatric Oncology, and Pathology Services, Hospital U. Virgen de la Arrixaca, Murcia, Spain; Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (Ciberehd). Molecular Genetics, Hospital U. Son Dureta, Palma de Mallorca, Spain; Inmunology Service, Hospital Gregorio Marañón, Madrid, Spain

* To whom correspondence should be addressed. Email: alfredo.minguela{at}carm.es.


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Abstract

We report the first case of hemophagocytic lymphohistiocytosis (HLH) induced by the monoclonal expansion of EBV-negative NK-cells. Consanguinity of parents made it necessary to discard familial HLH in patient and HLA-identical sister and demonstrate that no cause other than the expansion of NK-cells, highly secretory of IFN-{gamma}, was the inducer.