Objective: To analyze the clinical characteristics, treatment, and prognosis of adult patients with early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL).
Methods: Clinical data of 113 T lymphoblastic leukemia/lymphoma (T-ALL/LBL) patients from January 2006 to January 2019 were collected from three hematology research centers, including Peking University Third Hospital, the First Medical Center of Chinese PLA General Hospital and Institute of Hematology and Blood Diseases Hospital, Chinese Medical University. The clinical characteristics and prognosis of ETP-ALL/LBL patients were analyzed compared with non-ETP-ALL/LBL patients.
Results: In 113 T-ALL/LBL patients, 13 cases (11.5%) were diagnosed as ETP-ALL/LBL, including 11 males, with a median age of 28(18-53) years. Compared with non-ETP-ALL/LBL patients, there were no significant differences in age, sex, incidence of large mediastinal mass, clinical stage, international prognostic index (IPI) score, white blood cell (WBC) count and lactate dehydrogenase (LDH) level among ETP-ALL/LBL patients. Among 13 ETP-ALL/LBL patients, 9 cases (69.2%) achieved complete remission (CR), and there was no statistically significant difference in response rate induced by chemotherapy between ETP-ALL/LBL patients and non-ETP-ALL/LBL patients. Among patients who received chemotherapy without allogeneic hematopoietic stem cell transplantation (allo-HSCT), ETP-ALL/LBL group had a worse 5-year overall survival (OS) rate compared with non-ETP-ALL/LBL group (0 vs 7.1%, P =0.008), while in patients with allo-HSCT, there was no significant difference for 5-year OS rate between the two group (37.5% vs 40.2%, P >0.05). Multivariate Cox regression analysis showed that CR after induction therapy, allo-HSCT, and LDH level were independent prognostic factors affecting T-ALL/LBL patients.
Conclusion: No significant difference in response rate induced by chemotherapy is observed between ETP-ALL/LBL and non-ETP-ALL/LBL patients. Allo-HSCT consolidation after induction of remission therapy may have significant favorable influence on OS for patients with ETP-ALL/LBL.
题目: 多中心成人早期前体T细胞白血病/淋巴瘤的临床特征及预后研究.
目的: 对3个血液学中心的成人早期前体T细胞白血病/淋巴瘤（ETP-ALL/LBL）患者进行回顾性分析，总结其临床特点、治疗及预后影响因素。.
方法: 收集2006年1月至2019年1月来自北京大学第三医院、解放军第一医学中心和中国医学科学院血液学研究所3个血液研究中心共113例T淋巴母细胞白血病/淋巴瘤（T-ALL/LBL）患者的临床数据资料，对其中ETP-ALL/LBL及非ETP-ALL/LBL患者的临床特征及预后进行分析比较。.
结果: 113例T-ALL/LBL患者 中，13例诊断为ETP-ALL/LBL（11.5%），其中男性患者11例（84.6%），中位年龄28（18-53）岁。与非ETP-ALL/LBL患 者相比，ETP-ALL/LBL患者在年龄、性别、纵隔大包块发生率、临床分期、IPI评分、白细胞水平、乳酸脱氢酶水平方面差异无统计学意义。在13例ETP-ALL/LBL患者中，9例（69.2%）获得完全缓解，ETP-ALL/LBL患者较非ETP-ALL/LBL患者化疗诱导缓解率无统计学差异。在单纯化疗未进行异基因造血干细胞移植的患者中，ETP-ALL/LBL组较非ETP-ALL/LBL组显示出更差的5年生存率（0vs 7.1%，P =0.008），而在进行异基因造血干细胞移植的患者中，两组5年生存率无统计学差异（37.5% vs 40.2%，P >0.05）。多因素Cox回归分析提示，诱导治疗达到完全缓解、异基因造血干细胞移植以及乳酸脱氢酶水平为影响T-ALL/LBL的独立预后因素。.
结论: ETP-ALL/LBL较其他类型T-ALL/LBL患者诱导化疗反应率无显著差异，诱导缓解后续贯异基因造血干细胞移植巩固治疗对于提高ETP-ALL/LBL患者远期生存率具有重要意义。.
Keywords: T lymphoblastic leukemia/lymphoma; prognosis; early T-cell precursor acute lymphoblastic leukemia/lymphoma; prognosis.