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目的探讨儿童急性早幼粒细胞白血病(APL)的治疗、长期生存和预后因子。方法对该院1998年4月至2005年10月收治的APL患儿46例进行临床分析。诱导缓解采用全反式维甲酸(ATRA)+柔红霉素(DNR)或吡柔吡星(THP),巩固治疗采用大剂量阿糖胞苷与DA(柔红霉素+阿糖胞苷)、HA(高三尖杉酯碱+阿糖胞苷)、EA(足叶乙甙+阿糖胞苷)方案交替给药,维持阶段ATRA与DA,HA,EA方案交替治疗,总疗程为2.5年。结果39例患儿进行了正规治疗,36例(92.3%)获完全缓解(CR)。39例患儿1年、3年和5年总体生存率(OS)分别为(86.1±5.8)%,(76.1±7.5)%和(70.2±8.9)%,1年、3年、5年的无事件生存率(EFS)分别是(78.4±6.8)%,(63.6±8.7)%和(53.1±10.0)%。5年累积复发率(CIR)28.6%。其中WBC≤10.0×109/L者的5年OS[(81.4±10.3)%]明显高于WBC>10.0×109/L者[(51.6±14.7)%,P<0.05]。获CR的PML/RARα融合基因短型(S型)的患儿5例最后全部死亡,而长型(L型)患儿13例无死亡发生,两者差异有统计学意义(P<0.01)。结论ATRA+DNR或THP诱导缓解治疗儿童APL安全、有效,可以作为初发儿童APL的标准诱导治疗方案。联合蒽环类及阿糖胞苷等药物化疗能明显改善长期生存率。高WBC和S型PML/RARα融合基因阳性的患儿预后不佳。
Objective To investigate the treatment, long-term survival and prognosis of childhood acute promyelocytic leukemia (APL). Methods A total of 46 APL children admitted to our hospital from April 1998 to October 2005 were analyzed. ATRA + DNR or THP were used in the induction of remission. Consolidation therapy consisted of high doses of cytarabine and DA (daunorubicin + cytarabine) , HA (homoharringtonine + cytarabine) and EA (etoposide + cytarabine) were alternately administered. During the maintenance phase, the alternative treatment of ATRA and DA, HA and EA regimens was performed with a total duration of 2.5 years . Results Thirty-nine children underwent formal treatment and 36 patients (92.3%) achieved complete remission (CR). The overall survival rates at one year, three years and five years in 39 children were (86.1 ± 5.8)%, (76.1 ± 7.5)% and (70.2 ± 8.9)%, respectively Event-free survival (EFS) were (78.4 ± 6.8)%, (63.6 ± 8.7)% and (53.1 ± 10.0)%, respectively. 5-year cumulative recurrence rate (CIR) 28.6%. The 5-year OS [(81.4 ± 10.3)%] of WBC≤10.0 × 109 / L was significantly higher than that of WBC> 10.0 × 109 / L [(51.6 ± 14.7)%, P <0.05]. All of the 5 patients with short form (S type) of PML / RARα fusion gene were finally killed, while 13 patients with long type (L type) had no death. The difference was statistically significant (P <0.01) . Conclusions ATRA + DNR or THP-induced remission is safe and effective in children with APL. It can be used as a standard induction therapy for APL in new-onset children. Combined anthracycline and cytarabine chemotherapy can significantly improve long-term survival. Poor WBC and S-type PML / RARα fusion gene-positive children with poor prognosis.