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.
Keywords: Lymphoblastic lymphoma; T cells; clinical features; gene mutation; prognosis.
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