BCR-ABL基因阳性成人急性淋巴细胞白血病29例临床分析

Clinical analysis of 29 cases BCR-ABL-positive adult acute lymphoblastic leukemia

目的总结BCR-ABL融合基因阳性的急性淋巴细胞白血病(acute lymphoblastic leukemia,ALL)患者的临床特点、疗效和预后。方法回顾分析29例确诊为BCR-ABL基因阳性的ALL患者,予传统化疗、伊马替尼和异基因造血干细胞移植治疗,随访期3～80月,评价治疗效果。结果178例ALL患者中,BCR-ABL基因阳性者29例(16.3%),其中B细胞性28例,T细胞性者1例。14例患者在首1～2个疗程化疗后获得完全缓解(48.3%),部分缓解者1例(3.4%),2例在首2个疗程未获CR后加用伊马替尼获得骨髓细胞学完全缓解(10.3%),无效12例(41.4%)。29例患者中位生存期14.5月。伊马替尼联合传统化疗化疗者完全缓解率80%,高于单用传统化疗者(50%()χ2=5.894,P<0.01),生存期(18.6月)大于单用传统化疗未进行移植者(11.1月)(t=2.469,P<0.05)。结论BCR/ABL融合基因阳性的ALL患者传统化疗疗效差,联合伊马替尼可提高完全缓解率和生存期,异基因造血干细胞移植可使部分患者长期无病生存。

Objective To study the clinical characteristics,therapeutic outcome and prognostic of BCR-ABL-positive adult acute lymphoblastic leukemia.Methods The clinical data of 29 newly diagnosed adults with BCR-ABL（＋）ALL were retrospectively analyzed.The follow-up period were 3～80 months.Results There were 29（16.1%） of 178 ALL patients diagnosed as BCR-ABL（＋）ALL.Among the 29 cases,28 patients（96.6%） were classified as B immunophenotype and 3.4% as T immunophenotype.The complete remission（CR） rate after 1 or 2 cycles of induction chemotherapy was 48.3%.Part remission rate was 3.4%,2 cases（18.3%） obtained CR when treated by combination imatinib and traditional chemotherapy after failure of traditional chemotherapy alone.The median survival time of 29 cases was 14.5 months.For patients treated with imatinib combining traditional chemotherapy and those with traditional chemotherapy alone,CR rate was 80% and 50%（P〈0.01）,the median survival time was 18.6 months and 11.1 months（P〈0.05）.For 2 patients receiving allo-HSCT after CR,1 case overall survival was 19 months,another obtained long-term disease-free survival.Conclusion The BCR-ABL（＋） adult ALL had bad outcome of traditional chemotherapy.The combination of imatinib and traditional chemotherapy can improve CR rate and median survival time.was beneficial to improve the CR rate and survival duration.Part of patients treated by allo-HSCT after complete remission can obtain long-term disease-free survival.