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The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China

Early T-cell precursor (ETP) lymphoblastic leukemia/lymphoma is a high-risk T lymphoblastic leukemia/lymphoma (T-ALL/LBL) subgroup. We performed a real-world multicenter study to explore the clinical characteristics and prognosis of adolescent and young adults (AYA) and older adult ETP leukemia/lymp...

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Autores principales: Xiao, Jinyan, Cai, Zihong, Wang, Hao, Li, Xuekai, Zhou, Biqi, Liu, Yujie, Wang, Ying, Xu, Peipei, Wang, Li, Wu, Depei, Dou, Liping, Zhou, Hongsheng, Xu, Yang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9207171/
https://www.ncbi.nlm.nih.gov/pubmed/35734596
http://dx.doi.org/10.3389/fonc.2022.846573
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author Xiao, Jinyan
Cai, Zihong
Wang, Hao
Li, Xuekai
Zhou, Biqi
Liu, Yujie
Wang, Ying
Xu, Peipei
Wang, Li
Wu, Depei
Dou, Liping
Zhou, Hongsheng
Xu, Yang
author_facet Xiao, Jinyan
Cai, Zihong
Wang, Hao
Li, Xuekai
Zhou, Biqi
Liu, Yujie
Wang, Ying
Xu, Peipei
Wang, Li
Wu, Depei
Dou, Liping
Zhou, Hongsheng
Xu, Yang
author_sort Xiao, Jinyan
collection PubMed
description Early T-cell precursor (ETP) lymphoblastic leukemia/lymphoma is a high-risk T lymphoblastic leukemia/lymphoma (T-ALL/LBL) subgroup. We performed a real-world multicenter study to explore the clinical characteristics and prognosis of adolescent and young adults (AYA) and older adult ETP leukemia/lymphoma. A total of 103 patients with ETP-ALL/LBL in five centers in China between January 2016 and February 2021 were included in this study. The median age was 29 years (range, 15–70 years). Next-generation sequencing was performed in 94 patients and revealed that NOTCH1 (35.1%, 33 cases) was the most frequently mutated gene, followed by JAK3 (16.0%, 15 cases), PHF6 (13.80%, 13 cases) and EZH2 (11.70%, 11 cases). Complete remission (CR) was obtained in 74.2% (72/97) of patients, and 6 relapsed/refractory patients received a decitabine combined with AAG priming regimen as reinduction therapy with a CR rate of 50%. With a median follow-up of 18 months (0.5–60 months), the 2-year overall survival (OS) and relapse-free survival (RFS) rates for the entire cohort were 54% and 57.7%, respectively. Allogeneic stem cell transplantation (allo-SCT) was performed in 59.8% (58/97) of patients. After landmark analysis at 6 months, the 2-year OS rates was 77% of patients with allo-SCT at CR1 and 25% of patients with chemotherapy alone (p < 0.001). A multivariate analysis suggested that allo-SCT and CR after the first course induction were independent prognostic factors for OS. Collectively, we reported the largest cohort study with AYA and older adult ETP-ALL/LBL, and we found that ETP-ALL/LBL was highly invasive and had a poor long-term prognosis. Allo-SCT could significantly improve ETP-ALL/LBL patient survival.
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spelling pubmed-92071712022-06-21 The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China Xiao, Jinyan Cai, Zihong Wang, Hao Li, Xuekai Zhou, Biqi Liu, Yujie Wang, Ying Xu, Peipei Wang, Li Wu, Depei Dou, Liping Zhou, Hongsheng Xu, Yang Front Oncol Oncology Early T-cell precursor (ETP) lymphoblastic leukemia/lymphoma is a high-risk T lymphoblastic leukemia/lymphoma (T-ALL/LBL) subgroup. We performed a real-world multicenter study to explore the clinical characteristics and prognosis of adolescent and young adults (AYA) and older adult ETP leukemia/lymphoma. A total of 103 patients with ETP-ALL/LBL in five centers in China between January 2016 and February 2021 were included in this study. The median age was 29 years (range, 15–70 years). Next-generation sequencing was performed in 94 patients and revealed that NOTCH1 (35.1%, 33 cases) was the most frequently mutated gene, followed by JAK3 (16.0%, 15 cases), PHF6 (13.80%, 13 cases) and EZH2 (11.70%, 11 cases). Complete remission (CR) was obtained in 74.2% (72/97) of patients, and 6 relapsed/refractory patients received a decitabine combined with AAG priming regimen as reinduction therapy with a CR rate of 50%. With a median follow-up of 18 months (0.5–60 months), the 2-year overall survival (OS) and relapse-free survival (RFS) rates for the entire cohort were 54% and 57.7%, respectively. Allogeneic stem cell transplantation (allo-SCT) was performed in 59.8% (58/97) of patients. After landmark analysis at 6 months, the 2-year OS rates was 77% of patients with allo-SCT at CR1 and 25% of patients with chemotherapy alone (p < 0.001). A multivariate analysis suggested that allo-SCT and CR after the first course induction were independent prognostic factors for OS. Collectively, we reported the largest cohort study with AYA and older adult ETP-ALL/LBL, and we found that ETP-ALL/LBL was highly invasive and had a poor long-term prognosis. Allo-SCT could significantly improve ETP-ALL/LBL patient survival. Frontiers Media S.A. 2022-06-06 /pmc/articles/PMC9207171/ /pubmed/35734596 http://dx.doi.org/10.3389/fonc.2022.846573 Text en Copyright © 2022 Xiao, Cai, Wang, Li, Zhou, Liu, Wang, Xu, Wang, Wu, Dou, Zhou and Xu https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Xiao, Jinyan
Cai, Zihong
Wang, Hao
Li, Xuekai
Zhou, Biqi
Liu, Yujie
Wang, Ying
Xu, Peipei
Wang, Li
Wu, Depei
Dou, Liping
Zhou, Hongsheng
Xu, Yang
The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China
title The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China
title_full The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China
title_fullStr The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China
title_full_unstemmed The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China
title_short The Clinical Characteristics and Prognosis of AYA and Older Adult ETP-ALL/LBL: A Real-World Multicenter Study in China
title_sort clinical characteristics and prognosis of aya and older adult etp-all/lbl: a real-world multicenter study in china
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9207171/
https://www.ncbi.nlm.nih.gov/pubmed/35734596
http://dx.doi.org/10.3389/fonc.2022.846573
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