摘要
目的:研究儿童BCR/ABL融合基因阳性急性淋巴细胞白血病临床特点、遗传学特征、治疗反应及转归。方法:分析2008年8月—2015年4月在山西省儿童医院血液科治疗的16例BCR/ABL融合基因阳性急性淋巴细胞白血病患儿的临床特点、遗传学特征、治疗反应及转归,比较其与同期住院的BCR/ABL融合基因阴性急性淋巴细胞白血病患儿,在年龄、性别、初诊时外周血白细胞绝对值、免疫分型、染色体、融合基因、诱导治疗第15天、第33天骨髓状态及转归等方面的异同。结果:BCR/ABL融合基因阳性组16例患者均为初诊患者,男性所占比例与同期住院BCR/ABL阴性组比较,差异无统计学差异;与BCR/ABL融合基因阴性组比较年龄偏大;初诊时外周血白细胞更高。16例患儿免疫分型均为B细胞型,融合基因均为BCR/ABLP190阳性,15例行染色体检查,8例为正常染色体,4例仅有Ph染色体异常,另3例还有次要染色体异常。16例均采用高危化疗方案治疗,泼尼松治疗反应、诱导治疗第15天骨髓常规、第33天骨髓、3个月MRD与BCR/ABL融合基因阴性组比较,差异有统计学意义(P<0.05)。随访1~79个月,3年无事件生存率为20%,而同期住院BCR/ABL阴性组为82%,差异有统计学意义。结论:儿童BCR/ABL阳性急性淋巴细胞白血病为儿童高危型白血病,化疗疗效差。伊马替尼等酪氨酸激酶抑制剂对于儿童BCR/ABL阳性急性淋巴细胞白血病治疗评价还有待进一步研究。
Objective：The study was purposed to analyze the clinical and genetic features, therapeutic response and prog- nosis of childhood acute lymphoblastic leukemia with BCR/ABL fusion gene transcript and to guide clinical practice. Methods： A total of 100 patients with limb fractures were enrolled in the study from September 2012 to December 2015. The patients were divided into two groups according to the intervention group： routine group and conventional group （ n = 50）. The conventional group was given routine functional exercise intervention, the conventional group on this basis to give early passive functional in- tervention. The quality of life was assessed by the activities of daily living（ADL）. The follow - up period was 6 months. The ac- tivity of the elbow, ulnar joint, knee joint and the quality of life before and after the intervention were observed. Results： Com- pared with BCR/ABL negative, BCR/ABL positive pediatric acute lymphoblastic leukemia patients had older age and higher white blood cell counts. All of 16 cases were B immunulogy. 8 of 15 patients had normal chromosome,4 had philadelphia chro- mosome, 3 had secondary aberrations. All of 16 eases received chemotherapy of high risk scheme. Compared with BCB/ABL negative ALL on the rate of CR on day 15, the MRD on day 33 and 3 months （ P 〈 0.05 ）. Follow - up period was 1 ~ 79 months,the 3 years event free survival was 20%, on the contrary, 82% in BCR/ABL negative podiatric acute lymphoblastic leukemia patients. Condusion：BCR/ABL positive pediatric acute lympholastic leukemia patients have older age, higher white blood cel,poor prognosis The advantage of tyrosine kinase inhibitor to BCR/ABL gene positive childhood acute lymphoblastic leukemia still need study.
出处
《临床医药实践》
2017年第5期323-326,共4页
Proceeding of Clinical Medicine
基金
山西省卫生和计划生育委员会科研课题(项目编号:201301037)
