摘要目的:探讨经急性淋巴细胞白血病（ALL）儿童方案治疗达到完全缓解（CR）和部分缓解（PR）的T淋巴母细胞淋巴瘤（T-LBL）患者应用造血干细胞移植巩固治疗的疗效及预后因素。方法:收集2013年1月至2017年1月于唐都医院血液病中心接受治疗的T-LBL患者的临床资料，将达到CR或PR的患者纳入研究，进行回顾性分析。结果:①48例患者接受了ALL儿童方案治疗，经2个疗程的诱导化疗后39例达CR，9例达PR。其中接受自体造血干细胞移植（auto-HSCT）者14例，接受异基因造血干细胞移植（allo-HSCT）者7例，21例患者移植后造血功能均顺利重建。②中位随访时间31（9~16）个月。3年总生存（OS）率为61.0%（95% CI 53.7%~68.3%），3年无进展生存（PFS）率为54.8%（95% CI 47.1%~62.2%）。③移植组和未移植组3年OS率分别为84.7%和42.8%（ P=0.006），两组3年PFS率分别为75.4%和38.9%（ P=0.004）。④auto-HSCT组与allo-HSCT组患者的OS率、PFS率差异均无统计学意义（ P值分别为0.320、0.597）。⑤骨髓侵犯、未接受造血干细胞移植是影响患者长期预后的独立危险因素[ HR=5.804（95% CI 1.140~29.549）， P=0.034； HR=5.871（95% CI 1.711~20.140）， P=0.005]。 结论:ALL儿童方案化疗序贯造血干细胞移植治疗T-LBL疗效确切，安全性好。auto-HSCT与allo-HSCT患者的OS率及PFS率差异均无统计学意义。骨髓侵犯、未接受造血干细胞移植是影响T-LBL患者长期预后的独立危险因素。

abstractsObjective:To discuss the effect and prognostic factors of hematopoietic stem cell transplantation (HSCT) in patients with T-lymphoblastic lymphoma (T-LBL) who have achieved complete remission (CR) and partial response (PR) after pediatric-like acute lymphoblastic leukemia (ALL) therapy.Methods:Basic information and clinical data of patients with T-LBL treated in the hematologic center of Tangdu Hospital from January 2013 to January 2017 were collected, and the patients who achieved CR/PR were included in this study and retrospectively analyzed.Results:①A total of 48 patients received pediatric-like ALL chemotherapy, among which 39 patients achieved CR and 9 patients achieved PR after 2 courses of induction chemotherapy. Auto-HSCT was performed in 14 cases and allo-HSCT in 7 cases, and the hematopoietic function of all 21 patients was successfully reconstructed after transplantation. ②The follow-up period was 9-61 months, with a median of 31 months. The 3-year overall survival (OS) rate was 61.0% (95% CI 53.7%-68.3%) , and the 3-year progression-free survival (PFS) rate was 54.8% (95% CI 47.1%-62.2%) . ③The 3-year OS rate of transplantation group was 84.7%, and that of non-transplantation group was 42.8%. Significant difference of OS rate was observed between the 2 groups ( P=0.006) . The 3-year PFS rate was 75.4% in transplantation group and 38.9% in non-transplantation group. Significant difference of the PFS rate between the two groups was observed ( P=0.004) . ④No difference of OS rate between auto-HSCT and allo-HSCT groups was observed ( P=0.320) , same as the PFS rate ( P=0.597) . ⑤Among the prognostic factors, bone marrow invasion and no HSCT are independent risk factors affecting the long-term prognosis of patients. The mortality rate of patients with bone marrow invasion is about 5.804 times higher than that of patients without bone marrow invasion, and the mortality rate of patients with HSCT is about 5.871 times higher than that of patients without HSCT. Conclusion:T-LBL received pediatric-like ALL chemotherapy and HSCT has definite curative effect with lower transplant-related mortality and more safety. In the transplantation group, there is no significant difference of OS and PFS rates between patients receiving auto-HSCT and patients receiving allo-HSCT. Moreover, bone marrow invasion and no HSCT are both independent risk factors for long-term prognosis of patients.