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目的探讨混合谱系白血病(MLL)基因重排阳性急性髓系白血病(AML)患者的临床特点、预后,并与同期MLL基因重排阴性AML患者进行比较。方法观察随访51例MLL基因重排阳性AML病例(非M3型),分析临床特征、细胞形态学、免疫表型、细胞遗传学、早期死亡(early death,ED)、CR率、复发率、总体生存率(overall survival,OS)、移植效果等,并与同期随机选择的51例MLL基因重排阴性AML病例(非M3型)进行比较。结果 (1)与对照组患者相比,MLL基因重排阳性患者WBC数、LDH、外周血原始细胞比例明显增高(P<0.05),FAB分型中M4/M5比例明显增高(P<0.05)。(2)MLL基因重排阳性AML组单核系统的表面标志CD14、CD64、CD15和CD11b的表达明显高于对照组(P<0.05)。(3)MLL基因重排阳性AML患者总缓解率51.0%,复发率42.3%。而对照组总缓解率72.5%,复发率18.9%。MLL基因重排阳性AML患者较对照组患者缓解率低,易复发(P<0.05);至随访截止时MLL基因重排阳性AML患者OS为32.3%,明显低于对照组(P<0.05);MLL基因重排阳性AML患者中,单纯化疗患者3年OS率为26.7%,移植患者3年OS率为60.0%,可见异基因外周血造血干细胞移植明显提高了OS率(P<0.05)。结论 MLL基因重排阳性AML在AML-M4/M5中发生率高,化疗效果差,易复发,预后差,异基因外周血造血干细胞移植可显著改善其生存率。
Objective To investigate the clinical characteristics and prognosis of patients with mixed lineage leukemia (MLL) gene rearrangement-positive acute myeloid leukemia (AML) and to compare with those with MLL gene rearrangement-negative AML in the same period. Methods Fifty-one patients with MLL gene rearrangement-positive AML (non-M3 type) were followed up and analyzed. Clinical characteristics, cell morphology, immunophenotype, cytogenetics, early death (ED), CR rate, Overall survival (OS), transplantation effect and so on, and compared with 51 cases of MLL gene rearrangement negative AML cases (non-M3 type) randomly selected in the same period. Results (1) Compared with the control group, the proportion of WBC, LDH and peripheral blood blasts in patients with positive MLL gene rearrangement was significantly increased (P <0.05), while the ratio of M4 / M5 in FAB genotype was significantly higher (P <0.05) . (2) The expression of CD14, CD64, CD15 and CD11b on the mononuclear system of MLL gene rearrangement-positive AML group was significantly higher than that of the control group (P <0.05). (3) The overall remission rate of MLL gene rearrangement positive AML patients was 51.0%, the recurrence rate was 42.3%. The control group, the total remission rate was 72.5%, the recurrence rate was 18.9%. The MLL gene rearrangement positive AML patients had a lower remission rate and relapse than the control group (P <0.05). The OS of MLL gene rearrangement positive AML patients was 32.3%, significantly lower than that of the control group (P <0.05) The 3-year OS rate of patients with MLL gene rearrangement positive was 26.7% and the 3-year OS rate was 60.0%. The allogeneic peripheral blood hematopoietic stem cell transplantation significantly improved the OS rate (P <0.05). Conclusion MLL gene rearrangement-positive AML is highly expressed in AML-M4 / M5. The chemotherapy effect is poor, relapse is easy, and the prognosis is poor. Allogeneic peripheral blood stem cell transplantation can significantly improve its survival rate.