CCLG-97方案治疗标危型急性淋巴细胞性白血病患儿疗效的随访分析

Therapeutic effectiveness of CCLG-97 protocol on standard-risk childhood acute lymphoblastic leukemia

目的对119例儿童标危型(SR)急性淋巴细胞性白血病(ALL)治疗的效果进行随访分析,探讨如何提高其生存率。方法119例SRALL采用CCLG97方案进行治疗。结果4周诱导治疗完全缓解(CR)率为97.4%;21例失访:1998年、1999年、2000年、2001年、2002年各年失访率为63%、14%、10%、8%、5%;1年、2年、3年、4年、5年、6年生存率分别为93.3%、90.2%、88.0%、85.0%、85.0%、85.0%;13例复发,总复发率为13.8%,9例单独骨髓复发中有5例与不规则治疗有关,占56%;2例为4周未CR者于2年内复发;2例为正规疗程中复发,1例为亚二倍体,1例免疫分型示T淋巴系表达;4例为单独中枢神经系统(CNSL)复发,复发率为4.3%;全部患儿无睾丸白血病(TL)发生,无第二肿瘤发生。死亡15例,病死率为12.6%,化疗相关死亡5例,占4.2%。结论加强管理与正规治疗是提高我国儿童ALL长期存活率的重要措施之一;须在提高检测手段的基础上调整临床分型标准,CCLG97方案在减少标危型ALL骨髓复发的同时,并未增加化疗相关死亡率;应积极开展大剂量氨甲蝶呤(HDMTX)的治疗,进一步探索其剂量、用法及个体化的应用。

Objective With the improvement of the diagnosis and treatment, the complete remission (CR) rate and the survival rate of childhood acute lymphoblastic leukemia have been increased in the recent 10 years. The objective of this study was to analyze the outcomes of 119 standard-risk childhood acute lymphoblastic leukemia (SR-ALL) patients, and explore how to improve the survival rate in ALL. Methods A total of 119 patients aged 14 months to 15 years were diagnosed as SR-ALL according to the Suggestion of Diagnosis And Treatment for Childhood Acute Leukemia-1993. Among them, seventy-nine were boys and 40 were girls. All of the patients were treated with the CCLG-97 protocol and were followed up for a period of 20~78 months. Results The complete remission rate reached 97.4% in four-week induction. Twenty-one patients were out of follow-up, comprising 63%, 14%, 10%, 8% and 5% of all subjects in 1998, 1999, 2000, 2001 and 2002, respectively. The overall survival rates were 93.3%, 90.2%, 88.0%, 85.0%, 85.0% and 85.0% in 1 year, 2 years, 3 years, 4 years and 5 years, respectively. Relapses occurred in 13 patients (13.8%). Among 9 isolated hematologic relapses, 5 patients (56%) were given irregular therapy, 2 did not reach CR within 4 weeks and relapsed 2 years later, 2 accepted regular therapy, 1 was of hypodiploidy and 1 T-ALL. Isolated central nervous system (CNS) relapse occurred in 4 patients (4.3%). Fifteen patients (12.6%) died, 5 of whom (4.2%) died of complications. Conclusion Reinforcing administration and regular therapy are important to improve the long-term survival rate in childhood ALL. The clinical classification should be adjusted with the improvement of diagnostic methods. CCLG-97 protocol decreased the rate of the relapses in SR-ALL and didn′t increase the rate of therapy-related death. High-dose methotrexate should be used in therapy and its dosage, usage and individualized therapeutic regimen should be further studied.