摘要
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
Objective: To investigate the clinical treatment results of combined Tretinoin-chemotherapy protocol and kinetics of PML-RAR α fusion gene in childhood acute promyelocytic leukemia(APL). Methods: Ten children with APL were involved in this study. Induction therapy was Tretinoin alone(6 cases),Tretinoin plus chemotherapy(3 cases) and arsenic trixode(1 case). Postremission therapy consisted of three consolidation courses with DA,MA or HA and a monthly maintenance therapy over 4-5 years. Monitoring of minimal residual disease was performed regularly by RT-PCR assay for PML-RAR α at differential clinical stages. Results: Clinical complete remission(CR) was obtained in 9 cases (90%).After a median follow-up of 42 months(14-156 months), the estimated 5-year event-free survival was (56± 16.5)%.Four cases relapsed at 14-42 months after achieving CR and 5 cases remained continuing CR. PML-RAR α fusion gene was positive in all cases at CR and turned negative gradually during consolidation and maintenance treatment. The duration of conversion to RT-PCR negative status varied from 6 to 42 months.Four patients who were persistent positive(2 cases) or converted to positive(2 cases) for PML-RAR α relapsed. Conclusion: Continuous negative RT-PCR results are associated with long-term disease-free survival and may be considered as potentially curative. RT-PCR assay for detection of PML-RAR αshould be performed regularly during post-remission period. The hematological relapse could potentially be averted through treatment modification according to molecular monitoring results of PML-RAR α.
出处
《医学研究生学报》
CAS
2004年第2期148-150,154,共4页
Journal of Medical Postgraduates
