Objective To explore the immunophenotype and clinical prognosis of acute myeloid leukemia (AML) patients with ASXL1 or TET2 positive.Methods Clinical data of 162 patients with primary diagnosis of AML (except M3) were collected in the First Affiliated Hospital of Bengbu Medical College from January 1,2019 to May 31, 2022, including 10 patients in the ASXL1 and TET2 double mutation (ASXL1+ TET2+ group), 26 patients in the ASXL1 mutation (ASXL1+ group), 25 patients in the TET2 mutation (TET2+ group) and 121 patients without ASXL1 or TET2 mutations (ASXL1-TET2 double negative group). The immunophenotype and prognosis of the four groups were retrospectively analyzed to compare the complete remission (CR) rate, median progression-free survival (PFS) and median overall survival (OS). Results There were no statistically significant differences in age, gender, white blood cell count, red blood cell count, hemoglobin, platelet count and FAB typing among the four groups. Compared with the ASXL1- TET2- double negative group, ASXL1+ group and TET2+ group had a higher rate of abnormal chromosome karyotype (P=0.046) and a lower bone marrow blast cell count at the time of the initial diagnosis (P=0.037). Patients in the ASXL1+ group had lower CD7 (P=0.024), CD33 (P=0.043) and CD38 (P=0.023), CD34(P=0.011) antigen expression than the double negative group. The first CR rate in ASXL1+TET2+ group (P=0.007), ASXL1+ group (P=0.001) and TET2+ group (P=0.020) was significantly lower than double negative group, and the total CR rate in ASXL1+TET2+ group was significantly lower than double negative group (P=0.023).The median PFS and OS in the double negative group were 30 and 35 months, respectively, both of which were significantly prolonged compared with those in the ASXL1+ TET2+ group (P=0.002 and ＜0.001), the ASXL1+ group (P=0.015 and 0.005) and the TET2+ group (P=0.015 and 0.005). The median OS of ASXL1+TET2+ group was significantly shorter than that of the TET2+ group (P=0.019), and there was a trend towards shorter OS compared with the ASXL1+ group (P=0.055). Conclusion ASXL1 is an indicator of poor pr