成人Ph+急性淋巴细胞白血病不同BCR-ABL转录本特征及预后比较

目的 比较成人Ph+急性淋巴细胞白血病(ALL)不同融合基因转录本临床特征的差异,并探讨其与预后的关系.方法 回顾性分析1996年1月至2007年12月我院诊断为Ph+ALL患者(年龄≥15岁)的资料,比较两个BCR-ABL转录本组间的临床特征,探讨两组患者预后的差异.结果 (1)106例患者中位年龄34岁,中位白细胞数28.5×109/L.p190组67例,p210组35例,p210组较p190组中位年龄偏大(40岁、31岁,P=0.002),初诊血小板数高(49.5×109/L、31.5×109/L,P=0.012),中、重度脾大的发生率高(48.6%、25.4%,P=0.019).(2)两组患者完全缓解(CR)率分别为92.2%(59/64)和93.9%(31/33).(3)p190组中位总生存(OS)和中位无复发生存(RFS)分别为13个月和10个月;p210组中位OS和RFS分别为15个月和10个月.两组之间比较差异无统计学意义(P>0.05).结论 成人Ph+ALL中BCR-ABL以p190表达为主;p210患者较p190具有年龄偏大,初诊血小板数偏高,中、重度脾大发生率高的特点;两组在CR率、RFS和OS上无明显差异.

The role of BCR/ABL isoforms in the presentations and outcome of Philadelphia-positive acute lymphoblastic leukemia in adult patients

Objective To investigate the difference of clinical characteristics and outcomes between different isoforms of BCR/ABL in adults with Philadelphia-positive acute lymphoblastic leukemia (ALL).Methods The data of 106 adults with Ph+ALL diagnosed in our hospital from January 1, 1996 to December 31, 2007 were reviewed. The difference of clinical characteristics between different subgroups of BCR/ABL was compared and their relation with outcomes was studied. Results The median age of the 106 patients was 34 years and the median white blood cell count at baseline was 28. 5 × 109/L. Comparative analysis demonstrated that patients in p210 group had an older age, higher blood platelet count (BPC) and more frequent occurrence of splenomegaly. Referring to the outcomes, the complete remission (CR) rate of the two groups were 92. 2% and 93.9%, respectively. The median overall survival (OS) and relapse free survival (RFS) in p190 group were 13 months and 10 months, the 1,3-year estimated OS were (54. 7±6. 7)% and (5.5±5.2)%, and the 1,3-year estimated RFS were (40. 2±6. 8)% and (7. 8±6. 7)%,while in p210 group, the median OS and RFS were 15 months and 10 months, respectively, the 1,3-year estimated OS were (65.8±8. 9)% and (14. 5±7.4)%, and the 1,3-year estimated RFS were (48. 3±9. 4)% and (12. 9±7. 7)%. All of the above data had no statistic significance between the two groups.Conclusion Majority of the adults with Ph+ALL is p190 positive and patients with p210 have older age, higher BPC and more frequent occurrence of splenomegaly, while there is no significant difference between p190 group and p210 group in CR rate, RFS and OS.