Patients with SET-NUP214 fusion gene-positive acute leukemia are mainly young and middle-aged males, most of whom are diagnosed with T-cell acute lymphoblastic leukemia (T-ALL). Immunophenotyping is often accompanied by the expression of myeloid markers such as CD13 and CD33. Karyotype analysis presents normal karyotypes in some patients and abnormalities, including 9q34 deletion, in others. This type of leukemia is frequently accompanied by mutations of PHF6, NOTCH1, JAK3, and other genes. In terms of treatment, conventional chemotherapy has a low remission rate and tends to induce drug resistance, while allogeneic hematopoietic stem cell transplantation can bring benefits to some patients. The expression level of SET-NUP214 can be used as an indicator for follow-up of minimal residual disease and prognostic monitoring, but further expansion of sample size is required for verification to achieve better detection results.