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Tγδ LGLL identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients
Tγδ large granular lymphocyte leukemia (LGLL) is a rare variant of T-cell LGLL (T-LGLL) that has been less investigated as compared with the more frequent Tαβ LGLL, particularly in terms of frequency of STAT3 and STAT5b mutations. In this study, we characterized the clinical and biological features...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The American Society of Hematology
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10163282/ https://www.ncbi.nlm.nih.gov/pubmed/36096473 http://dx.doi.org/10.1182/blood.2021013489 |
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author | Barilà, Gregorio Grassi, Angela Cheon, HeeJin Teramo, Antonella Calabretto, Giulia Chahal, Jasmanet Vicenzetto, Cristina Almeida, Julia Shemo, Bryna C. Shi, Min Gasparini, Vanessa Rebecca Munoz-Garcia, Noemi Pastoret, Cédric Nakazawa, Hideyuki Oshimi, Kazuo Sokol, Lubomir Ishida, Fumihiro Lamy, Thierry Orfao, Alberto Morice, William G. Loughran, Thomas P. Semenzato, Gianpietro Zambello, Renato |
author_facet | Barilà, Gregorio Grassi, Angela Cheon, HeeJin Teramo, Antonella Calabretto, Giulia Chahal, Jasmanet Vicenzetto, Cristina Almeida, Julia Shemo, Bryna C. Shi, Min Gasparini, Vanessa Rebecca Munoz-Garcia, Noemi Pastoret, Cédric Nakazawa, Hideyuki Oshimi, Kazuo Sokol, Lubomir Ishida, Fumihiro Lamy, Thierry Orfao, Alberto Morice, William G. Loughran, Thomas P. Semenzato, Gianpietro Zambello, Renato |
author_sort | Barilà, Gregorio |
collection | PubMed |
description | Tγδ large granular lymphocyte leukemia (LGLL) is a rare variant of T-cell LGLL (T-LGLL) that has been less investigated as compared with the more frequent Tαβ LGLL, particularly in terms of frequency of STAT3 and STAT5b mutations. In this study, we characterized the clinical and biological features of 137 patients affected by Tγδ LGLL; data were retrospectively collected from 1997 to 2020 at 8 referral centers. Neutropenia and anemia were the most relevant clinical features, being present in 54.2% and 49.6% of cases, respectively, including severe neutropenia and anemia in ∼20% of cases each. Among the various treatments, cyclosporine A was shown to provide the best response rates. DNA samples of 97 and 94 cases were available for STAT3 and STAT5b mutation analysis, with 38.1% and 4.2% of cases being mutated, respectively. Clinical and biological features of our series of Tγδ cases were also compared with a recently published Tαβ cohort including 129 cases. Though no differences in STAT3 and STAT5b mutational frequency were found, Tγδ cases more frequently presented with neutropenia (P = .0161), anemia (P < .0001), severe anemia (P = .0065), and thrombocytopenia (P = .0187). Moreover, Vδ2(−) cases displayed higher frequency of symptomatic disease. Overall, Tγδ cases displayed reduced survival with respect to Tαβ cases (P = .0017). Although there was no difference in STAT3 mutation frequency, our results showed that Tγδ LGLL represents a subset of T-LGLL characterized by more frequent symptoms and reduced survival as compared with Tαβ LGLL. |
format | Online Article Text |
id | pubmed-10163282 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | The American Society of Hematology |
record_format | MEDLINE/PubMed |
spelling | pubmed-101632822023-05-07 Tγδ LGLL identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients Barilà, Gregorio Grassi, Angela Cheon, HeeJin Teramo, Antonella Calabretto, Giulia Chahal, Jasmanet Vicenzetto, Cristina Almeida, Julia Shemo, Bryna C. Shi, Min Gasparini, Vanessa Rebecca Munoz-Garcia, Noemi Pastoret, Cédric Nakazawa, Hideyuki Oshimi, Kazuo Sokol, Lubomir Ishida, Fumihiro Lamy, Thierry Orfao, Alberto Morice, William G. Loughran, Thomas P. Semenzato, Gianpietro Zambello, Renato Blood Lymphoid Neoplasia Tγδ large granular lymphocyte leukemia (LGLL) is a rare variant of T-cell LGLL (T-LGLL) that has been less investigated as compared with the more frequent Tαβ LGLL, particularly in terms of frequency of STAT3 and STAT5b mutations. In this study, we characterized the clinical and biological features of 137 patients affected by Tγδ LGLL; data were retrospectively collected from 1997 to 2020 at 8 referral centers. Neutropenia and anemia were the most relevant clinical features, being present in 54.2% and 49.6% of cases, respectively, including severe neutropenia and anemia in ∼20% of cases each. Among the various treatments, cyclosporine A was shown to provide the best response rates. DNA samples of 97 and 94 cases were available for STAT3 and STAT5b mutation analysis, with 38.1% and 4.2% of cases being mutated, respectively. Clinical and biological features of our series of Tγδ cases were also compared with a recently published Tαβ cohort including 129 cases. Though no differences in STAT3 and STAT5b mutational frequency were found, Tγδ cases more frequently presented with neutropenia (P = .0161), anemia (P < .0001), severe anemia (P = .0065), and thrombocytopenia (P = .0187). Moreover, Vδ2(−) cases displayed higher frequency of symptomatic disease. Overall, Tγδ cases displayed reduced survival with respect to Tαβ cases (P = .0017). Although there was no difference in STAT3 mutation frequency, our results showed that Tγδ LGLL represents a subset of T-LGLL characterized by more frequent symptoms and reduced survival as compared with Tαβ LGLL. The American Society of Hematology 2023-03-02 2022-09-14 /pmc/articles/PMC10163282/ /pubmed/36096473 http://dx.doi.org/10.1182/blood.2021013489 Text en © 2023 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Lymphoid Neoplasia Barilà, Gregorio Grassi, Angela Cheon, HeeJin Teramo, Antonella Calabretto, Giulia Chahal, Jasmanet Vicenzetto, Cristina Almeida, Julia Shemo, Bryna C. Shi, Min Gasparini, Vanessa Rebecca Munoz-Garcia, Noemi Pastoret, Cédric Nakazawa, Hideyuki Oshimi, Kazuo Sokol, Lubomir Ishida, Fumihiro Lamy, Thierry Orfao, Alberto Morice, William G. Loughran, Thomas P. Semenzato, Gianpietro Zambello, Renato Tγδ LGLL identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients |
title | Tγδ LGLL identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients |
title_full | Tγδ LGLL identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients |
title_fullStr | Tγδ LGLL identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients |
title_full_unstemmed | Tγδ LGLL identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients |
title_short | Tγδ LGLL identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients |
title_sort | tγδ lgll identifies a subset with more symptomatic disease: analysis of an international cohort of 137 patients |
topic | Lymphoid Neoplasia |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10163282/ https://www.ncbi.nlm.nih.gov/pubmed/36096473 http://dx.doi.org/10.1182/blood.2021013489 |
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