Objective To investigate the effect of NPM1 mutation on the prognosis of IDH2-mutated acute myeloid leukemia patients.Methods A total of 105 AML patients admitted to the Department of Hematology of The First Affiliated Hospital of Bengbu Medical College from January 2018 to June 2020 were selected. AML patients were divided into IDH2 mutation positive group (28 cases) and IDH2 mutation negative group (77 cases). The differences in CR rate and 2-year survival rate were compared between the two groups. Results 1. Among the 105 AML patients, 56 patients were not accompanied by NPM1 mutation, of which 15 cases were IDH2 mutation positive and 41 cases were IDH2 mutation negative. The CR rate was 33.3% (5/15) in 15 patients with positive IDH2 mutation and 70.7% (29/41) in 41 patients with negative IDH2 mutation. The difference between the two groups was statistically significant (P<0.05). 2. The 2-year survival rate of 15 patients with positive IDH2 mutation was 40.0%(6/15) and the median OS was 17.00 months. The 2-year survival rate of 41 patients with negative IDH2 mutation was 70.7%(29/41), and the median OS was 24.00 months. There were significant differences in survival rate between the two groups (P<0.05). Conclusion Under the background that AML was not accompanied by NPM1 mutation, the chemotherapy efficacy and 2-year survival rate of patients with positive IDH2 mutation are worse than those with negative IDH2 mutation