Clinical Study Of Pediatric Core Binding Factor-Acute Myeloid Leukemia

OBJECTIVE:The aim is to analyze the clinical and genetic characteristics of pediatric core binding factor-acute myeloid leukemia(CBF-AML),and to explore the association of prognosis with additional cellular molecular genetic events in this disease.METHODS:From January 2016 to September 2018,44 children with CBF-AML were admitted to our center.Clinical grouping and statistical analysis were performed according to clinical features and laboratory tests.The survival time was followed up and compared between the groups.Kaplan-Meier estimation was used to map survival curves.Log-rank test was used to compare the statistical differences between survival curves to explore the association between additional genetic abnormalities and children’ s survival.RESULTS:Of the 44 pediatric cases with CBF-AML,35 had fusion gene AML1-ET0 and 9 had fusion gene CBF β-MYH11.The CBF β-MYH11 group had a higher initial white blood cell count(45.41×109/L vs 13.69×109/L,P=0.015)and the proportion of newly diagnosed peripheral blood leukemia cells(56.0%vs 37.0%,P<0.001).The most common cytogenetic abnormality in children with CBF-AML is sex chromosome deletion,the incidence rate is 45.5%(20/44),and both-X and-Y occur only in the AML1-ET0 group(P=0.566,0.016),but there was no significant effect on the prognosis of the disease.The most common additional molecular genetic abnormality in this disease is KIT mutation,with a total incidence of 48.8%(21/43).KIT,c-KIT8 and c-KIT17 mutations compared to KIT wild type showed poor relapse-free survival(RFS),but the difference was not statistically significant.CONCLUSION:Pediatric CBF-AML fusion gene subtypes have different performances in clinical laboratory examination and cell molecular genetic abnormalities.Compared with cases without KIT mutations,children with positive KIT mutations in CBF-AML may have a poorer prognostic tendency.