| Objective:T-ALL/LBL is a rare type of adult hematological malignancy with highly heterogeneous characters.The outcome of T-ALL/LBL is unfavorable.So far,a few clinical studies have explored the efficacy and prognostic factors of T-ALL/LBL in adults patients.However,the results were controversy.The purpose of this study was to demonstrate the clinical features of adult T-ALL/LBL patients in our center,and to analyze the factors that influence the efficacy and prognosis of patients with T-ALL/LBL.Methods:We retrospectively collected fifty T-ALL/LBL patients aged over 18 years who were admitted to the Department of Hematology in the First Hospital of Jilin University from January 1,2009 to June 30,2018.A total of 41 T-ALL patients and 9T-LBL patients were included.Relevant clinical data were collected which including gender,age at diagnosis,white blood cell level at the time of initial diagnosis,immunophenotype,cytogenetic risk,induction regimens,CR(or CRi)after induction therapy,MRD after induction therapy and allo-HSCT.Results:1.Clinical features: Among the fifty patients,33 patients were male and other 17 patients were female.The ratio of male to female was 1.9:1.The median age at diagnosis was 31(range,18 to 70)years old.Thirty-seven patients(74.0%)aged <40years old and 4 patients(8.0%)aged > 60 years old.At the initial diagnosis,13patients(26.0%)had high white blood cells.The median WBC count was 9.16(range,0.23 to 681.49)×109/L.The median hemoglobin was 103.5(range,45.0 to 152.0)g/L and the median platelet count was 73.5(range,4.0 to 415.0)×109/L.The median bone marrow blast count was 79.5%(range,0 to 99.0).The results of genetic risk stratification were 45 patients in the good prognosis group and 5 patients in the poor prognosis group.A total of 24 patients had genetic changes,accounting for 48.0%.There were 9 patients with T-LBL,and all of them had superficial lymphadenopathy.Seven cases were in stage IV,1 case was in stage III and 1 case was in stage II,respectively.There were 7 cases with large mediastinal mass,accounting for 77.8%.2.Efficacy and overall survival: Overall response rate(CR + CRi)after induction chemotherapy was 56.0%(28 of the 50 patients).The median follow-up period was 18.0(range,0.8 to 77.1)months.The median OS was 20.0(95% CI13.722~26.344)months and the median EFS was 11.3(95% CI 10.032~12.501)months.The recurrence rate was 50.0%.The 2-year OS rate and EFS rate were 40.6%and 33.5%,respectively.3.Analysis of prognostic factors: The univariate analysis showed that the factors affecting OS and EFS were CR after induction therapy,MRD after induction therapy and allo-HSCT.For patients who did not reach CR after induction therapy,there was a statistically significance in OS and EFS between the allo-HSCT group and the chemotherapy group(P=0.002,P=0.031).Multivariate analysis showed that the age at diagnosis,CR after induction therapy(HR=1.786,95% CI 0.539~5.915,P=0.342),MRD after induction therapy(HR=1.962,95% CI 0.650~5.921,P=0.232)and allo-HSCT(HR=1.780,95% CI 0.660~4.796,P=0.254)were not independent prognostic factors affecting OS in adult T-ALL/LBL patients.CR after induction therapy(HR=3.409,95% CI 1.224~9.500,P=0.019)was a favorable prognostic factor affecting EFS in T-ALL/LBL.Conclusion:1.T-ALL/LBL occurrred mostly in young men.Most of T-LBL patients were in stage III/IV and with mediastinal mass at the time of initial diagnosis.In our center,ETP-ALL/LBL patients accounted for a higher proportion in T-ALL/LBL patients.2.This study indicated that CR after induction therapy was an independent prognostic factor affecting EFS in adult T-ALL/LBL patients.And CR after induction therapy,MRD after induction therapy and allo-HSCT were prognostic factors affecting OS and EFS,but not independent factors.3.The age at initial diagnosis still be probable factors affecting prognosis of adult patients with T-ALL/LBL.4.Allo-HSCT could significantly improve the prognosis of these patients who did not reach CR after induction chemotherapy. |
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