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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.
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-
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-
associated with hemophagocytic lymphohistiocytosis
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, was the inducer.
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