Familial hemophagocytic lymphohistiocytosis: a model for understanding the human machinery of cellular cytotoxicity |
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| Authors: | Elena?Sieni,Valentina?Cetica,Elena?Mastrodicasa,Daniela?Pende,Lorenzo?Moretta,Gillian?Griffiths,Maurizio?Aricò mailto:m.arico@meyer.it" title=" m.arico@meyer.it" itemprop=" email" data-track=" click" data-track-action=" Email author" data-track-label=" " >Email author |
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| Affiliation: | (1) Dipartimento Oncoematologia Pediatrica e Cure Domiciliari, Azienda Ospedaliero-Universitaria Meyer, Viale Pieraccini, 24, 50139 Florence, Italy;(2) S.C. di Oncoematologia Pediatrica con Trapianto di CSE, Ospedale “S.M. della Misericordia” A.O, Perugia, Italy;(3) A.O.U. San Martino-IST, Istituto Nazionale Ricerca sul Cancro, Genoa, Italy;(4) IRCCS Istituto Giannina Gaslini, Genoa, Italy;(5) Cambridge Institute for Medical Research, Addenbrooke’s Hospital, Cambridge, CB2 0XY, UK; |
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| Abstract: | ![]()
Cytotoxic T lymphocytes, natural killer cells, and NKT cells are effector cells able to kill infected cells. In some inherited human disorders, a defect in selected proteins involved in the cellular cytotoxicity mechanism results in specific clinical syndromes, grouped under the name of familial hemophagocytic lymphohistiocytosis. Recent advances in genetic studies of these patients has allowed the identification of different genetic subsets. Additional genetic immune deficiencies may also induce a similar clinical picture. International cooperation and prospective trials resulted in refining the diagnostic and therapeutic approach to these rare diseases with improved outcome but also with improved knowledge of the mechanisms underlying granule-mediated cellular cytotoxicity in humans. |
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