We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Familial hemophagocytic lymphohistiocytosis: a model for understanding the human machinery of cellular cytotoxicity.
- Authors
Sieni, Elena; Cetica, Valentina; Mastrodicasa, Elena; Pende, Daniela; Moretta, Lorenzo; Griffiths, Gillian; Aricò, Maurizio
- 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.
- Subjects
BLOOD disease treatment; CELL-mediated cytotoxicity; KILLER cells; T cells; CLINICAL trials; GENETIC mutation; HEALTH outcome assessment
- Publication
Cellular & Molecular Life Sciences, 2012, Vol 69, Issue 1, p29
- ISSN
1420-682X
- Publication type
Article
- DOI
10.1007/s00018-011-0835-y