Objective:T cell lymphoblastic leukemia/lymphoma(T-ALL/LBL)is a rare but highly aggressive disease.Allogeneic hematopoietic stem cell transplantation(allo-HSCT)is though to be an effective way to cure high risk T-ALL/LBL.At present,there are limited data on the results of allo-HSCT in patients with T-ALL/LBL,especially for patients with high-risk(HR)characteristics.In addition,factors affecting the prognosis of T-ALL/LBL in adults are unknown.Therefore,this study aimed to retrospectively analyze the treatment outcomes of 56 HR T-ALL/LBL patients who underwent alloHSCT,and to explore the factors affecting survival and prognosis.Method:In this study,we retrospectively analyzed the treatment outcomes of 56 T-ALL/LBL patients with HR features who underwent allo-HSCT at Zhujiang Hospital of Southern Medical University between January 2005 and September 2022.According to the clinical data and follow-up results of patients,the occurrence of graft-versus-host disease(GVHD),overall survival(OS),leukemia-free survival(LFS),cumulative incidences of relapse(CIR),non-relapse mortality(NRM)and risk factors for prognosis were assessed.Result:A total of 56 patients were included in this study,and engraftment were successful in 55(98.0%)patients.The cumulative incidence of aGVHD grade ⅡⅣ was 42.9%,and the cumulative incidence of cGVHD was 46.4%.The median follow-up for this study was 36.6 months(range 10.3 to 142.3 months).As of the last follow-up,a total of 22(39.3%)patients relapsed after allo-HSCT and 26(46.4%)patients died.The 2-year OS and LFS of this study were 60.3%and 57.1%,respectively.The 2-year CIR was 35.8%and the 2-year NRM was 14.7%.Univariate analysis showed that the involvement of central nervous system(CNS)at diagnosis significantly affected OS(HR,10.60;95%CI,3.32-33.81;p=0.001),LFS(HR,9.17;95%CI,2.9128.88;p=0.004)and CIR(HR,19.07;95%CI,4.53-80.24;p=0.001).The status of minimal residual disease(MRD)at reduce remission also had a significantly impact on OS(HR,3.26;95%CI,1.39-7.63;p=0.01),LFS(HR,3.05;95%CI,1.30-7.15;p=0.003)and CIR(HR,4.17;95%CI,1.61-10.79;p=0.003).The OS,LFS,CIR,NRM were significantly influenced by disease status at transplantation.The 2-year OS was 78.3%and LFS was 75.7%in patients who underwent allo-HSCT in CR1,compared with 35.7%and 28.6%for those who underwent allo-HSCT in≥CR2(p<0.001 for both).In addition,patients who underwent allo-HSCT in≥CR2 had a higher CIR and NRM compared with those in CR1(2-year CIR 57.2%vs 25.0%,p=0.01;2-year NRM 36.5%vs 3.7%,p=0.001).The 2-year OS and LFS were lower in patients without cGVHD compared with those who develop cGVHD(OS 40.0%vs 83.4%,p<0.001;LFS 36.7%vs 80.8%,p<0.001).Multivariate analysis showed that CNS involvement at diagnosis(HR,14.76,95%CI,2.88-75.59;p=0.001)and the disease status at transplantation in≥CR2(HR,5.79;95%CI,1.83-18.31;p=0.003)were independent risk factors for OS,while the development of cGVHD after allo-HSCT was an independent protective factor for OS(HR,0.15,95%CI,0.44-0.54;p=0.003).Independent risk factors for LFS were the disease status at transplantation in≥CR2(HR,3.13;95%CI,1.02-9.63;p=0.05)and the development of cGVHD after alloHSCT as an independent protective factor for LFS(HR,0.21;95%CI,0.07-0.69;p=0.01).The independent protective factor for CIR was the development of cGVHD after transplantation(HR,0.20;95%CI,0.06-0.71;p=0.01).The disease status at transplantation in≥CR2 was also an independent risk factor for NRM(HR,13.92;95%CI,1.60 to 120.98;p=0.02).Conclusion:Allo-HSCT was an effective way of treating HR T-ALL/LBL patients.The disease status at transplantion and the development of cGVHD after transplation were independent prognostic factors affecting the therapy.For HR T-ALL/LBL patients,we should try to make patients reach CR status and perform allo-HSCT as soon as possible.The development of low-grade cGVHD after transplantation is beneficial to reduce disease recurrence. |