MLL-AF10阳性急性白血病的临床特征及预后分析

OBJECTIVE: To analyze the clinical features and prognosis of acute leukemia patients with the mixed lineage leukemia (MLL) gene rearrangements AF10 positive. METHODS: 6 cases with MLL-AF10 positive were analyzed retrospectively, related literatures were reviewed to clarify MLL-AF10 patients' clinical features and prognosis. RESULTS: The median age of 6 cases was 19.5 years old, 5 patients with fever onset, the onset white blood cells of 4 patients were less than 10×10(9)/L. 5 cases were as M(5) and 1 case M(4) according to FAB classification, the level of fusion gene (RQ-PCR) was 0.23%–22.60% when diagnosed, 4 cases had concomitant WT1 gene mutation, flow cytometry disclosed myeloid phenotype. Of 5 evaluated patients achieved the first complete remission after conventional chemotherapy, 2 cases of complex karyotype died, one case died of sepsis in induction, another died from failing of transplantation. 4 out of 5 transplant recipients gained long term survival. CONCLUSION: The MLL-AF10 positive patients were mostly young men, the majority FAB classification was M(5) or M(4), often onset with fever, low white blood cells and low level of fusion gene, usually associated with WT1 mutation. Conventional chemotherapy produced a high response rate, but easy to relapse, while the complex karyotype had a poor prognosis, allo-HSCT may have the potential to improve the prognosis of MLL-AF10 positive patients.