Our meta-analysis showed that IDH inhibitor monotherapy has a good prognosis in R/R AML patients with IDH mutations and could be another option for R/R AML patients with IDH mutations (CR 21%, ORR 40%, median OS: 8.21 months, median EFS: 8.21 months, 2-year OS rate: 15%). This evidence concerns the gene IDH2 and acute myeloid leukemia.