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目的 :研究儿童急性早幼粒细胞白血病 (APL)的临床治疗和PML RARα 融合基因连续检测的意义。 方法 :以全反式维A酸 (ATRA)单用或联合化疗进行诱导缓解 ,常规联合化疗巩固 ,常规化疗、小剂量化疗和维A酸 (Tretinoin)交替维持治疗的方案 ,治疗 10例儿童APL。应用逆转录聚合酶链扩增 (RT PCR)方法在病程的不同阶段连续检测PML RARα变化 ,作为鉴测微小残留白血病细胞的指标。 结果 :10例APL中完全缓解 (CR) 9例 ,CR率 90 %。 9例CR患儿中 4例在CR后 14～ 4 2个月复发 ;1例仍在继续治疗中 ;生存期达 34个月 ;4例在连续CR 4～ 5年后已停药 ,停止治疗时间为 18～ 96个月 ,生存期达 72～ 15 6个月。 10例患儿中 ,8例在病程中PML RARα 转为阴性 ,首次转阴时间为 6～ 4 2个月 ,1例持续阳性。4例复发患儿中 ,2例复发前持续阳性 ,2例为病程中由阴性转为阳性。 5例仍生存者 ,至少已 2次连续检查为阴性。 1例在病程中由阴性转为阳性、2例分别在持续CR 36和 4 2个月仍阳性的患儿 ,在治疗干预后均转阴 ,且长期生存。结论 :在连续CR期定期检测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。持续PML RARα阴性的分子缓解是APL治愈的保证
Objective: To study the clinical treatment of children with acute promyelocytic leukemia (APL) and the significance of continuous detection of PML RARα fusion gene. Methods: All-trans retinoic acid (ATRA) alone or combined with chemotherapy for induction of remission, conventional chemotherapy and consolidation, conventional chemotherapy, low-dose chemotherapy and Tretinoin alternately maintenance treatment program, the treatment of 10 children with APL . The changes of RARα in PML were detected continuously by reverse transcription-polymerase chain reaction (RT-PCR) at different stages of the course of disease, and used as an indicator to detect minimal residual leukemia cells. Results: In 10 APLs, 9 cases were completely relieved (CR) and the CR rate was 90%. 4 of 9 CR patients relapsed 14 to 42 months after CR; 1 patient was still continuing treatment; the survival time was 34 months; 4 patients discontinued after 4 to 5 years of continuous CR and stopped treatment Time for 18 to 96 months, the survival period of 72 to 15 6 months. Of the 10 children, 8 had negative PML RARα during the course of the disease, with a first negative conversion of 6 to 42 months and 1 persistent positive. Of the 4 children with recurrent disease, 2 were persistent positive before recurrence and 2 were negative to positive during the course of disease. Five patients were still alive, at least 2 consecutive negative examination. One patient turned negative to positive during the course of disease, and two patients were still positive after continuous CR 36 and 42 months, respectively, and both were negative and long-term survival after treatment intervention. CONCLUSIONS: Regular detection of PML RARα in consecutive CR can detect molecular recurrence early, and timely intervention can prevent hematological relapse. Persistent PML RARα negative molecular remission is an assurance of APL cure