| ObjectiveTo retrospectively analyze the clinical features,prognostic risk factors and therapeutic effects of 74 patients with acute lymphoblastic leukemia(ALL)treated by TOTXV regimen of St.Jude Research Hospital in our hospital.MethodA retrospective analysis was performed on 74 children with ALL who were initially diagnosed and finished treatment in our hospital from January 2015 to December 2022.Clinical characteristics,Overall survival OS and Event-free survival of children with acute lymphoblastic leukemia treated by Total XV program of St.Jude Research Hospital in the United States EFS),Relapse-free survival RFS.ResultFollow-up as of December 31,2022,the median follow-up time was 55 months(1-97months).There were 42 males and 32 females,with a median age of 4.2(0.33-14.08)years.There were 5 cases(6.8%)in the low-risk group,50 cases(67.6%)in the medium-risk group,and 19 cases(25.7%)in the high-risk group.Acute T-lymphoblastic leukemia(T-ALL)in 2 cases(2.7%),acute B-lymphoblastic leukemia(B-ALL)in 72 cases(97.3%),There were 2 cases(2.7%)of early precursor T-lymphocytic leukemia(Pro-T-ALL)and 61 cases(82.4%)of Common B-cell lymphocytic leukemia(Common-B-ALL).There were 2 cases(2.7%)of pre-B-cell lymphocytic leukemia and 9 cases(12.2%)of early Pro-B-ALL leukemia.32 cases(43.2%)had abnormal chromosomes and 42 cases(56.8%)had normal chromosomes.There were 31 cases(41.9%)with no gene mutation,4 cases(5.4%)with MLL/AF4 fusion gene,17 cases(23%)with TEL/AML fusion gene,and 22 cases(29.7%)with other gene mutation.At D19 days,31 cases(41.9%)were negative,42 cases(56.8%)were positive,and 1 case was deleted.At D26 days,MRD was negative in 21 cases(28.4%),positive in 21 cases(28.4%),and deleted in 32 cases.At day 46,61 patients(82.4%)were negative and 10 patients(13.5%)were positive.There were 7 deaths and7 relapses,including 6 cases of bone marrow recurrence(85.7%)and 1 case of extramedullary recurrence(14.2%).Analysis of risk factors for death in children: Age<1 year old,age ≥ 1 year old and<10 years old,age>10 years old.The 5-year OS of the three groups was 57.1%,93.2%,and 100%,respectively,p=0.002.The 5-year OS of the low-risk group,medium risk group,and high-risk group were 100%,100%,and 63.2%,respectively,with P<0.001.The 5-year OS of T-ALL group and B-ALL group were 50% and 91.7%,respectively,with P=0.013.The 5-year OS of the Pro-T-ALL,Common-B-ALL,Pro-B-ALL,and Pre-B-ALL groups were 50.0%,96.7%,55.6%,and 100%,respectively,with P<0.001.The 5-year OS of the D19 day MRD negative group and D19 day MRD positive group were 100% and 83.3%,respectively,with P=0.018.The5-year OS of the D46 day MRD negative group and the D46 day MRD positive group were 96.7% and 60%,respectively,with P<0.001.The 5-year OS of the recurrent group and the non recurrent group were 97% and 28.6%,respectively,with P<0.001.No gene mutation,MLL/AF4 fusion gene,TEL/AML gene fusion,and other gene mutations were found in 90.3%,50.0%,100%,and 90.9% of the 5-year OS,respectively,with P=0.015.The risk of death in the recurrent group was 42.67 times higher than that in the non recurrent group [95% CI(2.963-614.407),P=0.006],and the difference was statistically significant.Recurrence is an independent risk factor for death.Analysis of recurrence risk factors: 5-year RFS of low-risk group,medium-risk group and high-risk group were 100%,95.5% and 71.1%,respectively,P< 0.05 with statistical difference.The 5-year OS of T-ALL group and B-ALL group were 50% and91.7%,respectively,P=0.016.The 5-year RFS of Pro-T-ALL,Common-B-ALL,Pre-B-ALL and Pro-B-All groups were 50%,93.0%,100% and 71.4%,respectively,P=0.025.The 5-year RFS of D19-day MRD negative group and D19-day MRD positive group were 100% and 82.3%,P =0.017.The 5-year RFS of D26-day MRD negative group and D26-day MRD positive group were 95.2% and 69.8%,respectively,P =0.04.The 5-year RFS of D46-day MRD negative group and D46-day MRD positive group were 94.5% and 60.0%,P < 0.05.Multivariate analysis of RFS showed that the risk of recurrence was 11.189 times higher in the D46-day MRD-positive group than in the D46-day MRD-negative group[95%CI(2.032-61.6),P=0.006].The risk of recurrence in the B-ALL group was 0.009 times that in the T-ALL group [95%CI(0-0.18),P=0.002],and the risk of recurrence in the B-ALL group was lower than that in the T-ALL group.Analysis of risk factors with EFS: Age < 1 year,age ≥1 year,age < 10 years,age >10 years,5-year EFS of the 3 groups were 42.9%,87.8%,100%,P< 0.001.The 5-year EFSof low-risk group,medium-risk group and high-risk group was 100%,93.6% and61.1%,P< 0.001,and the difference was statistically significant.EFS of T-ALL and B-ALL groups were 50.5% and 87.0%,respectively,P=0.049.The 5-year EFS of Pro-T-ALL group,Common-B-ALL group,Pro-B-ALL group and Pre-B-ALL group were 50.0%,89.7%,62.5% and 100%,respectively,P=0.025.The 5-year EFS of D26-day MRD negative group and D26-day MRD positive group were 95.2% and66.3%,P=0.024.The EFS of D46-day MRD negative group and D46-day MRD positive group were 89.6% and 60.0%,respectively,at 5 years,P=0.005.Analysis of overall survival rates: 5-year OS was 90.5%[95%CI(83.8-94.6%)],5-year RFS was 90.5%[95%CI(84.1-94.7%)],5-year EFS was 85.1%[95%CI(79.0%-91.7%)].Conclusion1.Age,risk,immune typing and MRD were the factors affecting the prognosis of children,and MRD at day D46 was directly related to recurrence,which was an independent risk factor for death.2.T-ALL children have poorer prognosis than B-ALL children.3.The Total XV regimen at St.Jude Research Hospital in the United States treats childhood ALL with a higher overall survival rate,with the adolescent group benefiting more. |
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