Cargando…
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...
Autores principales: | , , , , , , , , , , , , |
---|---|
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 |
_version_ | 1784729457321312256 |
---|---|
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. |
format | Online Article Text |
id | pubmed-9207171 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT xiaojinyan theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT caizihong theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT wanghao theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT lixuekai theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT zhoubiqi theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT liuyujie theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT wangying theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT xupeipei theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT wangli theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT wudepei theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT douliping theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT zhouhongsheng theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT xuyang theclinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT xiaojinyan clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT caizihong clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT wanghao clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT lixuekai clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT zhoubiqi clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT liuyujie clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT wangying clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT xupeipei clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT wangli clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT wudepei clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT douliping clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT zhouhongsheng clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina AT xuyang clinicalcharacteristicsandprognosisofayaandolderadultetpalllblarealworldmulticenterstudyinchina |