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26 results on '"Moretta L"'

1. Group 3 innate lymphoid cells regulate neutrophil migration and function in human decidua

Author

Croxatto, D., primary, Micheletti, A., additional, Montaldo, E., additional, Orecchia, P., additional, Loiacono, F., additional, Canegallo, F., additional, Calzetti, F., additional, Fulcheri, E., additional, Munari, E., additional, Zamò, A., additional, Venturini, P.L., additional, Moretta, L., additional, Cassatella, M.A., additional, Mingari, M.C., additional, and Vacca, P., additional

Published
2016
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13. HLA-G-specific receptors in human NK or T lymphocytes

Author

Schiavetti, F., primary, Pende, D., additional, Sivori, S., additional, Accame, L., additional, Pareti, L., additional, Ponte, M., additional, Le Bouteiller, P., additional, Moretta, L., additional, Mingari, M.C., additional, and Moretta, A., additional

Published
1997
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19. Human T lymphocytes expressing TCR gamma/delta

Author

Moretta, A., primary, Bottino, C., additional, Pende, D., additional, Tripodi, G., additional, Orengo, A.M., additional, Millo, R., additional, Pelicci, P.G., additional, Ciccone, E., additional, and Moretta, L., additional

Published
1990
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26. Genetic predisposition to hemophagocytic lymphohistiocytosis: Report on 500 patients from the Italian registry

Author

Franco Locatelli, Lorenzo Moretta, Concetta Micalizzi, Valentina Cetica, Elena Sieni, Carmen De Fusco, Franca Fagioli, Cesare Danesino, Maria Caterina Putti, Maurizio Aricò, Andrea Biondi, Lucio Luzzatto, Gillian M. Griffiths, Daniela Pende, Griffiths, Gillian [0000-0003-0434-5842], Apollo - University of Cambridge Repository, Cetica, V, Sieni, E, Pende, D, Danesino, C, De Fusco, C, Locatelli, F, Micalizzi, C, Putti, M, Biondi, A, Fagioli, F, Moretta, L, Griffiths, G, Luzzatto, L, and Aricò, M

Subjects
0301 basic medicine, Male, PRF1, Hemophagocytic, immunologic tests, Disease, Hemophagocytic lymphohistiocytosis, HSCT, Hematopoietic stem cell transplantation, FHL, Familial hemophagocytic lymphohistiocytosis, NK, Natural killer, immunologic test, Immunology and Allergy, Registries, Child, Lymphohistiocytosis, biology, Familial Hemophagocytic Lymphohistiocytosis, Middle Aged, 3. Good health, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Italy, Child, Preschool, Female, Hemophagocytosis, Hemophagocytic lymphohistiocytosi, Adult, Adolescent, Immunology, Lymphohistiocytosis, Hemophagocytic, 03 medical and health sciences, Young Adult, XLP, X-linked lymphoproliferative syndrome, UNC13D, Humans, Infant, Infant, Newborn, Membrane Proteins, Perforin, Genetic Predisposition to Disease, Immune Deficiencies, Infection, and Systemic Immune Disorders, medicine, Genetic predisposition, Preschool, business.industry, medicine.disease, Newborn, HLH, Hemophagocytic lymphohistiocytosis, 030104 developmental biology, Macrophage activation syndrome, biology.protein, hemophagocytic lymphohistiocytosis, MAS, Macrophage activation syndrome, business
Abstract

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 based on an analysis of our 25 years of experience. Methods From our registry, we have analyzed a total of 500 unselected patients with HLH. Results Biallelic pathogenic mutations defining FHL were found in 171 (34%) patients; the proportion of FHL was much higher (64%) in patients given a diagnosis during the first year of life. Taken together, mutations of the genes PRF1 (FHL2) and UNC13D (FHL3) accounted for 70% of cases of FHL. Overall, a genetic diagnosis was possible in more than 90% of our patients with FHL. Perforin expression and the extent of degranulation have been more useful for diagnosing FHL than hemophagocytosis and the cytotoxicity assay. Of 281 (56%) patients classified as having "sporadic" HLH, 43 had monoallelic mutations in one of the FHL-defining genes. Given this gene dosage effect, FHL is not strictly recessive. Conclusion We suggest that the clinical syndrome HLH generally results from the combined effects of an exogenous trigger and genetic predisposition. Within this combination, different weights of exogenous and genetic factors account for the wide disease spectrum that ranges from HLH secondary to severe infection to FHL.

Published
2016
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