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本研究分析急性白血病(AL)伴11q23/mll融合基因阳性的患者临床和分子生物学特点,探讨适宜的治疗策略。对36例急性白血病伴mll融合基因阳性患者的骨髓和外周血细胞形态学、免疫学、分子生物学、染色体特征以及患者治疗、生存情况进行总结分析。结果表明,在494例急性白血病患者中,mll融合基因阳性共36例,占7.2%,其中32例为急性髓细胞白血病(AML),免疫表型均为髓系表达,有5例同时伴淋系表达;4例为急性淋巴细胞白血病(ALL),免疫表型为B淋巴系表达,有3例同时伴有非系别确定性髓系标志表达。36例患者中29例(80%)可检测到克隆性染色体异常,其中有22例检测到11号染色体改变。对所有患者均进行了化疗,总缓解率为47.2%,25例缓解后的病人有10例在6个月内复发,其复发率为40%,有9例进行了异基因造血干细胞移植,其中7例移植后存活;共死亡24例。36例患者的中位生存期为16(2-46)个月,2年总生存率(OS)为41.4%,9例移植病人2年OS为87.5%。结论:白血病伴mll融合基因阳性的患者化疗效果差,易复发,预后差,异基因造血干细胞移植可明显改善MLL融合基因阳性AL患者的生存。
This study analyzed the clinical and molecular biological characteristics of acute leukemia (AL) patients with positive 11q23 / mll fusion gene and explored appropriate treatment strategies. 36 cases of acute leukemia with mll fusion gene positive patients with bone marrow and peripheral blood cell morphology, immunology, molecular biology, chromosome characteristics and patient treatment and survival were analyzed. The results showed that in 494 patients with acute leukemia, mll fusion gene was positive in a total of 36 cases, accounting for 7.2%, of which 32 cases of acute myeloid leukemia (AML), the immune phenotype are myeloid, 5 cases with concomitant leaching 4 cases were acute lymphoblastic leukemia (ALL), the immunophenotype was B lymphoid lineage, and 3 cases were accompanied by non-lineage definite myeloid lineage expression. Twenty-nine out of 36 patients (80%) could detect clonal chromosome abnormalities, of which 22 detected chromosome 11 change. Chemotherapy was given to all patients, with a total response rate of 47.2%; 10 of 25 patients relapsed within 6 months with a recurrence rate of 40% and 9 patients undergoing allogeneic hematopoietic stem cell transplantation Seven patients survived after transplantation; a total of 24 patients died. The median survival of 36 patients was 16 (2-46) months, 2 years overall survival (OS) was 41.4%, and 9 patients with 2-year OS were 87.5%. Conclusion: Patients with positive leukemia and mll fusion gene have poor response, easy to relapse and poor prognosis. Allogeneic hematopoietic stem cell transplantation can significantly improve the survival of AL patients with MLL fusion gene.