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目的 探讨跟踪检测急性早幼粒细胞白血病 (APL)特有的PML RARα融合基因在发现APL早期复发和指导APL临床治疗方面的意义。方法 1 0例APL患儿用全反式维甲酸 (ATRA)和 (或 )其它化疗药物进行诱导缓解、巩固治疗和维持治疗 ,并进行随访。在病程的不同阶段采集骨髓标本进行形态学检查 ,并应用RT PCR方法检测PML RARα融合基因。结果 随访时间为 1 4～ 1 5 6月 (中位时间 4 2月 ) ,5年无病生存率为 5 6 .0 %±1 6 .5 %。 1 0例APL患儿完全缓解 (CR)率为 90 % ,早期死亡 1例。 9例CR病人中 4例在CR后 1 4～ 4 2月复发 ,4例在连续完全缓解 4～ 5年后已停药 ,停止治疗时间为 1 8～ 96月。 1例CR ,仍在继续治疗中。 9例CR患儿中 ,8例在病程中PML RARα转为阴性 ,1例持续阳性。 4例复发病人中 ,2例复发前持续阳性 ,2例在病程中由阴性转为阳性。 5例仍生存的患儿中 ,1例在病程中PML RARα由阴性转为阳性 ,2例分别在持续完全缓解 36和 4 2月仍呈阳性 ,这 3例患儿经治疗干预后均转阴 ,且长期生存。结论 对APL患儿跟踪检测PML RARα可早期发现分子复发 ,及时干预治疗可避免血液学复发。
Objective To investigate the significance of follow-up detection of PML RARα fusion gene specific for acute promyelocytic leukemia (APL) in the early detection of APL and the clinical treatment of APL. Methods A total of 10 children with APL were treated with all-trans retinoic acid (ATRA) and / or other chemotherapeutic agents to induce remission, consolidate and maintain treatment and follow-up. Bone marrow samples were collected at different stages of the course of disease for morphological examination, and RT-PCR method was used to detect PML RARα fusion gene. Results The follow-up time ranged from 14 to 165 months (median, 42 months). The 5-year disease-free survival rate was 56.0% ± 16.5%. One hundred patients with APL had complete remission (CR) rate of 90% and early death in 1 case. Four of nine CR patients relapsed 14 to 42 months after CR. Four patients discontinued after four to five years of continuous complete remission and the treatment period was 18 to 96 months. 1 case of CR, is still continuing treatment. In 9 cases of CR children, 8 cases of PML RARα in the course of the disease turned negative, one case of persistent positive. Of the 4 recurrent patients, 2 were persistent positive before relapse and 2 were negative to positive during the course of the disease. Of the 5 surviving children, one had positive PML RARα in the course of the disease, and two remained positive in the continuous complete remission 36 and 42 respectively, all of the three children were negative after treatment , And long-term survival. Conclusion The follow-up detection of PML RARα in children with APL can detect molecular recurrence early, and timely intervention can prevent hematological relapse.