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[Ph 阳性急性淋巴细胞白血病的预后意义]

[Prognostic significance of Ph-positive acute lymphoblastic leukemia].

作者信息

Zhou Li, Hu Jiong, Chen Juan, Du Shenghong, Wang Aihua, You Jianhua, Wu Wen, Shen Zhixiang, Li Junmin

机构信息

Department of Hematology, Shanghai Institute of Hematology, Shanghai Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2014 Feb;35(2):109-13. doi: 10.3760/cma.j.issn.0253-2727.2014.02.011.

Abstract

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 transcripts 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 follow-up 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.

摘要

目的

探讨Ph阳性和/或BCR-ABL阳性急性淋巴细胞白血病(Ph⁺ALL)的预后意义。

方法

对72例Ph⁺ALL患者进行回顾性分析,以探究预后因素,包括性别、年龄、诊断时白细胞计数高、额外染色体异常、BCR-ABL转录本类型、基于伊马替尼的治疗、异基因造血干细胞移植(allo-HSCT)以及一个疗程诱导后的完全缓解(CR)情况对Ph⁺ALL患者预后的影响。

结果

72例患者的中位年龄为40.5(13 - 68)岁,38例患者接受了伊马替尼联合化疗。中位随访时间为11(0.2 - 96)个月,接受伊马替尼联合化疗患者的总CR率高于仅接受化疗的患者(97.4%对62.3%,P = 0.019)。诊断时白细胞计数高或额外染色体异常对CR率无影响。伊马替尼联合化疗组的2年总生存(OS)率和无病生存(DFS)率分别为(28.9±7.4)%和(25±7.4)%,高于化疗组(P < 0.001)。HSCT组的OS率显著高于非HSCT组[(61.1±11.5)%对(5.6±3.1)%,P < 0.001]。对OS的多因素预后分析显示,基于伊马替尼的治疗[风险比(RR)=0.413(95%置信区间0.237 - 0.721),P = 0.002]、allo-HSCT[RR = 0.175(95%置信区间0.075 - 0.3 .89),P = 0.000]以及一个疗程诱导后的CR[RR = 0.429(95%置信区间0.245 - 0.750),P = 0.003]对生存至关重要。

结论

allo-HSCT是Ph⁺ALL患者的最佳选择。基于伊马替尼的治疗可提高CR率、维持CR持续时间并降低复发率,从而增加HSCT的机会。伊马替尼改善了不适合HSCT的Ph⁺ALL患者的预后。

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