A Single-center Study Of APL-2016 Regimen For Acute Promyelocytic Leukemia In Children

Objective:To analysis the clinical efficacy and prognosis of APL-2016 regimen for acute promyelocytic leukemia in children.Methods:The clinical data of 36 APL children with newly diagnosed May 2016 to May 2019 and treated with APL-2016 regimen in Blood Tumor Center of Children's Hospital of Chongqing Medical University were analyzed retrospectively,and to summarize and analysis the clinical efficacy and prognosis.Results:The median age of 36 children of this group is 7 years and 9months,male/female ratio is 1.25/1.The main clinical manifestations are fever,infection,hemorrhage,anemia and bone marrow infiltratio,etc.Leukopenia,thrombocytopenia and moderate anemia were more common in peripheral blood,72.2% of children with infantile granulocytes in peripheral blood.The abnormal coagulation function and LDH elevation were more common,4 children's LDH>500 IU.Auer corpuscles were observed in 97.2% of children's in bone marrow promyelocytes.There were highly positive expression rate of immunophenotypic CD9?CD13?CD33?MPO?CD117.Chromosome examination was completed in 33 children,with 93.8% abnormal karyotypes.Among them,87.8% are t(15;17)classical karyotype,including9 cases of abnormal karyotype with classic and additional chromosomes,1cases are non-classical karyotype.All 36 children met genetic and molecular diagnostic criteria,that is t(15,17)positive ? PML-RAR?positive(FISH+PCR).The proportion of low,middle and high risk groups is 11.1%,27.8% and 61.1%,respectively.Three of the 36 children had relapsed,with no deaths or missed visits case.The rate of HCR after treatment was 94.4%,the 4 year's EFS rate and OS rate was 83.3% ±10.5% and 100%,respectively.The remission status of MRD monitoring after treatment in 36 children had no significant effect on the rate of 4years' s EFS(P>0.05).Compared with children who was without combined with DIC in first diagnosis,there was a significant decrease of 4year's EFS rate(P=0.002)in who was combined with DIC.The 4 year's EFS rate was significantly decrease in patients who with LDH>500IU compared with who with LDH ? 500IU(P=0.004).The treatment related adverse reactions mainly include differentiation syndrome,coagulation dysfunction,bone marrow suppression after chemotherapy,septicemia,pneumonia,liver function damage and myocardial damage.Conclusion:The survival situation of childhood APL treated with APL-2016 therapeutic regimen is better.The combination of DIC and(or)LDH>500IU at first diagnosis is a poor prognostic factor for the APL children.