Clinical features, mutational landscape and their prognostic impact in Chinese paediatric patients with T‐cell lymphoblastic lymphoma

T-cell lymphoblastic lymphoma (T-LBL) is an aggressive lymphoma that primarily affects children and young adults, and a comprehensive understanding of its molecular features is crucial for improving patient outcomes. In this study, 552 patients were included, with targeted next-generation sequencing of 262 lymphoma-associated genes performed on tumour samples from 119 patients. The associations between mutations and survival rates, as well as relapse and other clinical factors, were analysed. The results demonstrated that pleural effusion (PE) invasion was significantly associated with adverse event-free survival (EFS) and overall survival (OS) (p < 0.05). Additionally, we identified 92 genes with recurrent mutations, among which NOTCH1 (44%), FBXW7 (28%), PHF6 (11%), KRAS (10%) and NRAS (10%) were the most frequently altered. Patients with NOTCH1 mutations exhibited improved EFS and OS (p < 0.01), whereas those carrying CREBBP, PTEN and LYST mutations exhibited worse prognosis (p < 0.05). In conclusion, NOTCH1 mutations are associated with a favourable prognosis in paediatric T-LBL, while CREBBP, PTEN and LYST mutations, as well as PE invasion, are linked to poor prognosis. This study identifies key molecular and clinical factors in paediatric T-LBL progression, aiding high-risk patient identification and personalized treatment strategies.

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Publication Details

Journal
British Journal of Haematology
Published
2025-09-16
DOI
https://doi.org/10.1111/bjh.70097
Citations
1
Primary Topic
Lymphoma Diagnosis and Treatment
Type
article
Field-Weighted Citation Impact
1.47
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article

Clinical features, mutational landscape and their prognostic impact in Chinese paediatric patients with T‐cell lymphoblastic lymphoma

Runming Jin, Keyan Yang, Hui Gao, Ling Jin et al.
1 citations
British Journal of Haematology
Lymphoma Diagnosis and Treatment
1.47
article

Clinical features, mutational landscape and their prognostic impact in Chinese paediatric patients with T‐cell lymphoblastic lymphoma

Runming Jin, Keyan Yang, Hui Gao, Ling Jin, Y H Zhang, Jinzhong Xu, Ansheng Liu, Xiaomei Yang, Rong Liu, Wei Liu, Lihua Yang, Lirong Sun, Ying Liu, Yang Li, Yunpeng Dai, Qinlong Zheng, Lian Jiang, Yueping Jia, Mincui Zheng
article en
1 citations

Abstract

T-cell lymphoblastic lymphoma (T-LBL) is an aggressive lymphoma that primarily affects children and young adults, and a comprehensive understanding of its molecular features is crucial for improving patient outcomes. In this study, 552 patients were included, with targeted next-generation sequencing of 262 lymphoma-associated genes performed on tumour samples from 119 patients. The associations between mutations and survival rates, as well as relapse and other clinical factors, were analysed. The results demonstrated that pleural effusion (PE) invasion was significantly associated with adverse event-free survival (EFS) and overall survival (OS) (p < 0.05). Additionally, we identified 92 genes with recurrent mutations, among which NOTCH1 (44%), FBXW7 (28%), PHF6 (11%), KRAS (10%) and NRAS (10%) were the most frequently altered. Patients with NOTCH1 mutations exhibited improved EFS and OS (p < 0.01), whereas those carrying CREBBP, PTEN and LYST mutations exhibited worse prognosis (p < 0.05). In conclusion, NOTCH1 mutations are associated with a favourable prognosis in paediatric T-LBL, while CREBBP, PTEN and LYST mutations, as well as PE invasion, are linked to poor prognosis. This study identifies key molecular and clinical factors in paediatric T-LBL progression, aiding high-risk patient identification and personalized treatment strategies.

British Journal of HaematologyVol. 207(5)
Qingdao University (CN), Hebei Medical University (CN), Union Hospital (HK), Shandong University (CN), Capital Medical University (CN), Peking University (CN), Dalian University of Technology (CN), 302 Military Hospital of China (CN), Capital Institute of Pediatrics (CN), Fourth Hospital of Hebei Medical University (CN), Zhengzhou Children's Hospital (CN), Shandong Provincial Hospital (CN), Affiliated Hospital of Qingdao University (CN), Beijing Hospital (CN), Zhujiang Hospital (CN), Peking University People's Hospital (CN), Wuhan Union Hospital (CN), Hunan Children's Hospital (CN), Xi’an Children’s Hospital (CN), Beijing Children’s Hospital (CN), Jiangxi Provincial Children's Hospital (CN), Dian Diagnostics (China) (CN), Qilu Hospital of Shandong University (CN), Shandong First Medical University (CN), Union Hospital (CN), Huazhong University of Science and Technology (CN), Southern Medical University (CN)
Openalex Percentile: Top 15%
Lymphoma Diagnosis and Treatment
1.47
DOISourceOpenAlex
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