Retrospective analysis of clinical and molecular characteristics as prognostic factors in adult T-cell lymphoblastic lymphoma.

IF 3.6 3区医学 Q2 ONCOLOGY

American journal of cancer research Pub Date : 2024-12-15 eCollection Date: 2024-01-01 DOI:10.62347/ZWAM1063

Li Ma, Jing Wang, Jin Zhao, Meijing Zheng, Xiaolian Wen, Liping Su

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引用次数: 0

Abstract

Objective: To analyze the clinical characteristics and molecular biomarkers of adult T-cell lymphoblastic lymphoma (T-LBL) to identify prognostic factors, and to evaluate the efficacy of different chemotherapy regimens, providing a basis for optimizing treatment strategies for T-LBL.

Methods: A total of 89 Patients aged 18-72 years with T-LBL, confirmed via histopathological examination of lymph nodes, extranodal tissues, or bone marrow, were retrospectively included. Clinical data, treatment details, and mutational profiles were collected. Prognostic factors were assessed based on clinical and molecular characteristics, and the efficacy and safety of two chemotherapy regimens were compared. Descriptive statistics were used to analyze the disease spectrum.

Results: Most patients (84.00%) presented with advanced disease (stages III-IV). Mediastinal invasion was observed in 63 patients (70.80%), and 59 patients (66.30%) exhibited B symptoms. Bone marrow involvement occurred in 19 patients (21.20%), and bulky mediastinum (>10 cm) was present in 50 patients (56.18%). Mutations were detected in 29 patients, with NOTCH1 being the most frequently mutated gene, followed by PHF-6, JAK-1, JAK-3, IL-7R, and TP53. The complete response (CR) rate was 51.69%. The 3-year overall survival (OS) and progression-free survival (PFS) rates were 74.9% and 58.80%, respectively. Multivariate analysis identified female sex, lack of CR, and elevated lactate dehydrogenase (LDH) levels (>2× normal) as independent predictors of poor OS (58.25%). Chemotherapy regimens, LDH levels, and sex were independent prognostic factors for PFS (21.24%).

Conclusion: T-LBL is characterized by high-frequency gene mutations across multiple signaling pathways. Mediastinal invasion (70.80%) and extranodal involvement (39.33%) were prevalent in Chinese patients and were associated with poor prognosis. Combined assessment of clinical and molecular features allows for improved prognostic stratification and facilitates the development of targeted therapies for high-risk patients.

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成人t细胞淋巴母细胞淋巴瘤的临床和分子特征作为预后因素的回顾性分析。

目的：分析成人t细胞淋巴母细胞淋巴瘤（T-LBL）的临床特征及分子生物标志物，探讨影响预后的因素，并评价不同化疗方案的疗效，为优化T-LBL的治疗策略提供依据。方法：回顾性分析89例年龄18-72岁，经淋巴结、结外组织或骨髓病理检查证实的T-LBL患者。收集临床资料、治疗细节和突变概况。根据临床和分子特征评估预后因素，并比较两种化疗方案的疗效和安全性。采用描述性统计分析疾病谱。结果：大多数患者（84.00%）表现为晚期疾病（III-IV期）。纵隔侵犯63例（70.80%），B型症状59例（66.30%）。19例（21.20%）患者出现骨髓受累，50例（56.18%）患者出现纵隔肥大（bbb10 cm）。29例患者检测到突变，其中NOTCH1是最常见的突变基因，其次是PHF-6、JAK-1、JAK-3、IL-7R和TP53。完全缓解（CR）率为51.69%。3年总生存率（OS）和无进展生存率（PFS）分别为74.9%和58.80%。多因素分析发现，女性、缺乏CR和乳酸脱氢酶（LDH）水平升高（bbb20正常值的2倍）是不良OS的独立预测因素（58.25%）。化疗方案、LDH水平和性别是PFS的独立预后因素（21.24%）。结论：T-LBL具有跨多种信号通路的高频基因突变特征。纵隔侵犯（70.80%）和结外受累（39.33%）在中国患者中普遍存在，并与预后不良相关。临床和分子特征的综合评估可以改善预后分层，促进高风险患者靶向治疗的发展。

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来源期刊

American journal of cancer research ONCOLOGY-

自引率

3.80%

发文量

263

期刊介绍： The American Journal of Cancer Research (AJCR) (ISSN 2156-6976), is an independent open access, online only journal to facilitate rapid dissemination of novel discoveries in basic science and treatment of cancer. It was founded by a group of scientists for cancer research and clinical academic oncologists from around the world, who are devoted to the promotion and advancement of our understanding of the cancer and its treatment. The scope of AJCR is intended to encompass that of multi-disciplinary researchers from any scientific discipline where the primary focus of the research is to increase and integrate knowledge about etiology and molecular mechanisms of carcinogenesis with the ultimate aim of advancing the cure and prevention of this increasingly devastating disease. To achieve these aims AJCR will publish review articles, original articles and new techniques in cancer research and therapy. It will also publish hypothesis, case reports and letter to the editor. Unlike most other open access online journals, AJCR will keep most of the traditional features of paper print that we are all familiar with, such as continuous volume, issue numbers, as well as continuous page numbers to retain our comfortable familiarity towards an academic journal.