Ma Li, Wang Jing, Zhao Jin, Zheng Meijing, Wen Xiaolian, Su Liping
Department of Hematology, Cancer Hospital Affiliated to Shanxi Medical University/Shanxi Province Cancer Hospital/Shanxi Hospital Affiliated to Cancer Hospital, Chinese Academy of Medical Sciences Taiyuan 030013, Shanxi, China.
Department of Pathology, Cancer Hospital Affiliated to Shanxi Medical University/Shanxi Province Cancer Hospital/Shanxi Hospital Affiliated to Cancer Hospital, Chinese Academy of Medical Sciences Taiyuan 030013, Shanxi, China.
Am J Cancer Res. 2024 Dec 15;14(12):5851-5862. doi: 10.62347/ZWAM1063. eCollection 2024.
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.
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.
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%).
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.
分析成人T细胞淋巴母细胞淋巴瘤（T-LBL）的临床特征和分子生物标志物，以确定预后因素，并评估不同化疗方案的疗效，为优化T-LBL的治疗策略提供依据。
回顾性纳入89例年龄在18至72岁之间、经淋巴结、结外组织或骨髓组织病理学检查确诊为T-LBL的患者。收集临床资料、治疗细节和突变谱。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。
大多数患者（84.00%）表现为晚期疾病（III-IV期）。63例患者（70.80%）观察到纵隔侵犯，59例患者（66.30%）出现B症状。19例患者（21.20%）发生骨髓受累，50例患者（56.
