Genetic predisposition to hemophagocytic lymphohistiocytosis: Report on 500 patients from the Italian registry.

@article{Cetica2016GeneticPT,
  title={Genetic predisposition to hemophagocytic lymphohistiocytosis: Report on 500 patients from the Italian registry.},
  author={Valentina Cetica and Elena Sieni and Daniela Pende and Cesare Danesino and Carmen De Fusco and Franco Locatelli and Concetta Micalizzi and Maria Caterina Putti and Andrea Biondi and Franca Fagioli and Alessandro Moretta and Gillian M Griffiths and Lucio Luzzatto and M. Rita Aric{\`o}},
  journal={The Journal of allergy and clinical immunology},
  year={2016},
  volume={137 1},
  pages={188-196.e4}
}
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease affecting mostly children but also adults and characterized by hyperinflammatory features. A subset of patients, referred to as having familial hemophagocytic lymphohistiocytosis (FHL), have various underlying genetic abnormalities, the frequencies of which have not been systematically determined previously. OBJECTIVE This work aims to further our understanding of the pathogenic bases of this rare condition… CONTINUE READING

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