CAJ | 학술논문

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。
목적：탐토혼합보계백혈병（MLL）기인중배양성급성수계백혈병（AML）환자적림상특점、예후，병여동기MLL기인중배음성AML환자진행비교。방법관찰수방51례MLL기인중배양성AML병례（비M3형），분석림상특정、세포형태학、면역표형、세포유전학、조기사망（early death，ED）、CR솔、복발솔、총체생존솔（overall survival，OS）、이식효과등，병여동기수궤선택적51례MLL기인중배음성AML병례（비M3형）진행비교。결과（1）여대조조환자상비，MLL기인중배양성환자WBC수、LDH、외주혈원시세포비례명현증고（P＜0.05），FAB분형중M4/M5비례명현증고（P＜0.05）。（2） MLL기인중배양성 AML조단핵계통적표면표지 CD14、CD64、CD15화 CD11b적표체명현고우대조조（P＜0.05）。（3）MLL 기인중배양성 AML 환자총완해솔51.0%，복발솔42.3%。이대조조총완해솔72.5%，복발솔18.9%。MLL기인중배양성AML환자교대조조환자완해솔저，역복발（P＜0.05）；지수방절지시MLL기인중배양성AML환자OS위32.3%，명현저우대조조（P＜0.05）；MLL기인중배양성AML환자중，단순화료환자3년 OS솔위26.7%，이식환자3년 OS솔위60.0%，가견이기인외주혈조혈간세포이식명현제고료OS솔（P＜0.05）。결론 MLL기인중배양성AML재AML-M4/M5중발생솔고，화료효과차，역복발，예후차，이기인외주혈조혈간세포이식가현저개선기생존솔。
Objective To investigate the clinical characteristics and prognosis of acute myoloid leukimia cases with MLL gene rearrangement. Methods 51 de novo MLL gene rearrangement AML(non M3)cases were retrospectively reviewed. The clinical features, cytomorphology, immunophenotype, cytogenetics, early death, complete remission rate, recurrence rate, overall survival and response to allo-HSCT of these patients were compared with 51 cases without MLL gene rearrangement (control group) admitted in the same period. Results (1) Compared with control group, the WBC count, LDH levels, percentage of peripheral blood blast cells in patients with MLL gene rearrangement increased notably(P<0.05);otherwise, the percentage of M4/M5 in these patient was much higher according to FAB classification(P < 0.05). (2) The expression of monocytic antigens such as CD14,CD64,CD15,CD11b in patients with MLL gene rearrangement were much higer than control group (P<0.05). (3) The total CR rate and recurrence rate in AML patients with MLL gene rearrangement were 51.0% and 42.3%, while those for patients in control group were 72.5%and 18.9%, respectively. It can be seen that, compared with control group, the AML patients with MLL gene rearrangement gained lower CR rate and were easier to relapse (P<0.05). At the end of the follow-up, the OS in patients with MLL gene rearrangement was 32.3%, which was much lower than that in control group (P<0.05). Among the patients with MLL gene rearrangement, the cases who received allo-HSCT acquired 3-year OS of 60.0% vs. 26.7% in cases with simple chemotherapy, resulting in a higher OS obviously(P<0.05). Conclusions AML with MLL gene rearrangement is highly correlated with M4/M5, and the patients of which show poor response to chemotherapy, high recurrence rate and poor prognosis. Hematopoietic stem cell transplantation may be a reasonable treatment principle to improve these patients’ survival situation.