CAJ | 학술논문

目的探讨Ph染色体阳性(Ph+)和(或)BCR-ABL融合基因阳性急性淋巴细胞白血病(Ph+ALL)的预后影响因素.方法回顾性分析72例Ph+ALL患者资料.比较性别、年龄(以50岁为界)、诊断时高白细胞计数(＞ 30×109/L)、附加染色体异常、BCR-ABL转录本类型、伊马替尼治疗、异基因造血干细胞移植(allo-HSCT)和治疗1个疗程获得完全缓解(CR)等因素对患者疗效和生存的影响.采用COX回归模型进行多因素分析.结果 72例Ph+ALL患者均为B系表达,中位年龄40.5(13～68)岁,中位随访时间为11(0.2～96)个月.伊马替尼联合化疗组38例患者的总体CR率达97.4％,明显优于单纯化疗组(62.3％,P=0.019).初诊时高白细胞计数和合并附加染色体异常对CR率无明显影响(P=0.392和P=0.249)；伊马替尼联合化疗组患者的2年总生存(OS)率和无病生存(DFS)率分别为(28.9±7.4)％和(25.0±7.4)％,明显优于单纯化疗组[(3.4±3.4)％和(3.4±3.4)％,P=0.000]；allo-HSCT组患者4年OS率明显优于化疗组[(61.1±11.5)％对(5.6±3.1)％,P=0.000]；多因素分析显示伊马替尼治疗[RP=0.413(95％ CI 0.237～0.721),P=0.002]、allo-HSCT[RR=0.175 (95％ CI 0.075～0.389),P=0.000]和治疗1个疗程获得CR[RR=0.429(95％ CI 0.245～0.750),P=0.003]是影响OS的独立预后因素.结论 allo-HSCT是Ph+ALL患者的最佳治疗选择；尽早使用伊马替尼能够使患者及早获得并维持CR,减少复发,为患者接受allo-HSCT提供更多的机会；对无条件进行移植的患者,伊马替尼联合化疗能改善预后.
목적탐 토Ph염색체양성(Ph+)화(혹)BCR-ABL융합기인양성급성림파세포백혈병(Ph+ALL)적예후영향인소.방법 회고성분석72례Ph+ALL환자자료.비교성별、년령(이50세위계)、진단시고백세포계수(＞ 30×109/L)、부가염색체이상、BCR-ABL전록본류형、이마체니치료、이기인조혈간세포이식(allo-HSCT)화치료1개료정획득완전완해(CR)등인소대환자료효화생존적영향.채용COX회귀모형진행다인소분석.결과 72례Ph+ALL환자균위B계표체,중위년령40.5(13～68)세,중위수방시간위11(0.2～96)개월.이마체니연합화료조38례환자적총체CR솔체97.4％,명현우우단순화료조(62.3％,P=0.019).초진시고백세포계수화합병부가염색체이상대CR솔무명현영향(P=0.392화P=0.249)；이마체니연합화료조환자적2년총생존(OS)솔화무병생존(DFS)솔분별위(28.9±7.4)％화(25.0±7.4)％,명현우우단순화료조[(3.4±3.4)％화(3.4±3.4)％,P=0.000]；allo-HSCT조환자4년OS솔명현우우화료조[(61.1±11.5)％대(5.6±3.1)％,P=0.000]；다인소분석현시이마체니치료[RP=0.413(95％ CI 0.237～0.721),P=0.002]、allo-HSCT[RR=0.175 (95％ CI 0.075～0.389),P=0.000]화치료1개료정획득CR[RR=0.429(95％ CI 0.245～0.750),P=0.003]시영향OS적독립예후인소.결론 allo-HSCT시Ph+ALL환자적최가치료선택；진조사용이마체니능구사환자급조획득병유지CR,감소복발,위환자접수allo-HSCT제공경다적궤회；대무조건진행이식적환자,이마체니연합화료능개선예후.
Objective To explore the prognostic significance of Ph-positive and/or BCR-ABL positive acute lymphoblastic leukemia (Ph+ALL).Methods A retrospective analysis of 72 patients with Ph + ALL to probe prognostic factors including sex,age,high white cell counts at diagnosis,additional chromosome abnormality,BCR-ABL trsnscripts type,imatinib based therapy,allo-HSCT and complete remission(CR) after one-course induction on the outcomes of Ph+ALL patients.Results Of 72 patients with median age 40.5 (13-68)years,38 patients received imatinib plus chemotherapy.With median followup of 11 (0.2-96)months,total CR rate in patients receiving imatinib plus chemotherapy was higher than of patients receiving chemotherapy only (97.4％ vs 62.3％,P=0.019).High white blood counts at diagnosis or additional chromosome abnormality had no effects on CR rate.2-year overall survival (OS) and disease free survival (DFS) in imatinib plus chemotherapy group were (28.9±7.4)％ and(25±7.4)％,respectively,which were higher than those in chemotherapy group(P＜0.001).OS rate in HSCT group was significantly higher than that in non-HSCT group [(61.1 ±11.5)％ vs (5.6±3.1)％,P＜0.001].Multivariate prognostic analysis for OS showed that imatinib-based therapy [RR=0.413 (95％ CI 0.237-0.721),P=0.002],allo-HSCT [RR=0.175(95％ CI 0.075-0.389),P=0.000]and CR after one-course induction [RR=0.429(95％ CI 0.245-0.750),P=0.003] were of importance for survival.Conclusion allo-HSCT was an optimal choice for Ph+ALL patients.Imatinib-based therapy could increase CR rate,maintain CR duration and decrease relapse,resulting in more chance of HSCT.Imatinib improved the outcomes of Ph+ALL patients who were not eligible for HSCT.