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Background The prognosis for relapsed or refractory acute myeloid leukemia (RR-AML) in children is poor,and the preferred salvage chemotherapy is unclear.One regimen is cladribine,cytarabine,and granulocyte-colony stimulating factor (CLAG),but little is known about its efficacy and safety in children with RR-AML.Methods We enrolled RR-AML patients aged 0-18 years who received modified CLAG regimen for re-induction between July 1,2015 and April 1,2018,or conventional induction between August 1,2011 and April 1,2018.Patients were followed up to March 31,2019.Patients underwent allogeneic stem cell transplantation (allo-SCT) or chemotherapy after the induction of complete remission (CR).The CR rate,survival,and side effects were analyzed.Results The CR rate for induction was 66.7% after one cycle and 75.0% after two cycles of the CLAG regimen in 12 children.The nine children who received conventional chemotherapy had a CR rate of 22.2% after one cycle and 33.3% after two cycles (P =0.087 vs.CLAG).The 3-year event-free survival (EFS) of the CLAG group and the conventional treatment group were 44.4 ± 15.7% and 22.2± 13.8% (P=0.112).The 3-year overall survival of the two groups were 59.5 ± 16.2% and 22.2% ± 13.8% (P=0.057).The 3-year EFS for allo-SCT and chemotherapy after CLAG regimen was 66.7 ± 19.2% and 25.0 ± 21.7% (P =0.015).A single case of chemotherapy-related death was recorded.Conclusion Our data suggest a promising CR rate using CLAG salvage treatment in childhood RR-AML.Allo-SCT after CR may improve the long-term outcome in these patients.