SUMS99方案治疗儿童急性淋巴细胞白血病的长期疗效

Analysis of Long-term Efficacy of SUMS 99 Protocol in 56 Childhood Acute Lymphoblastic Leukemia Patients

【目的】回顾和探讨56例儿童急性淋巴细胞白血病(acute lymphoblastic leukemia,ALL)分型治疗的疗效和预后因素。【方法】回顾分析1999～2002年收治的56例儿童ALL,按中山医科大学99(SUMS99)方案的分型标准,标危型ALL(standard risk-ALL,SR-ALL)33例,中危型(middle risk-ALL,IR-ALL)12例,高危型ALL(high risk-ALL,HR-ALL)11例;所有患者按SUMS99方案进行治疗。【结果】56例患者4周内完全缓解(complete remission,CR)率SR-ALL组和IR-ALL为100%,而HR-ALL组为72.73%(8/11),6周才CR者3例。SR-ALL组早期缓解时间为(17.1±6.4)d,IR-ALL组早期缓解时间为(24.5±5.6)d,HR-ALL组早期缓解时间为(29.9±6.3)d。本组共死亡13例,死亡率为23.0%,其中死于骨髓复发者9例,死于感染者4例。本组目前无病存活共43例,其中SR-ALL组为90.9%(30/33),IR-ALL组为83.3%(10/12),HR-ALL组为27.3%(3/11)。SR-ALL组平均生存时间为75.2个月,IR-ALL组平均生存时间为56.8个月,HR-ALL组平均生存时间为25.3个月。2例HR-ALL患者进行组织相容性抗原全相合外周血干细胞移植,目前均无病存活。【结论】分型治疗儿童急性淋巴细胞白血病是达到高生存率和改善生存质量的重要手段,但本方案仍然未能解决HR-ALL的疗效,选择合适供者进行异基因造血干细胞移植可能是今后改善HR-ALL预后的方向。

[Objective] To review and explore the efficacy and predictions of 56 children with acute lymphohlastic leukemia （ALL） according to clinical type from 1999 to 2002. [Methods] Fifty-six cases could insist on chemotherapy, and all the patients were treated according to SUMS （SUN Yat-sen Medical University） 99 protocol and according to clinical type. [Results] The complete remission （CR） rate in 4 weeks of standard risk- ALL（SR-ALL）group （n=33）, middle risk-ALL （IR-ALL）group （n=12） and high risk-ALL （HR-ALL） group （n= 11） were 100%, 100%, and 72.7%, respectively. The CR rate in 6 weeks of HR-ALL group was 100%. The mean time of early CR rate in SR-ALL, IR-ALL, and HR-ALL were 17.1±6.4 d, 24.5±5.6 d, and 29.9±6.3 d, respectively. Thirteen patients were died, the rate of death was 23.0%, including 9 patients for bone marrow relapse and 4 patients for infections. Forty-three patients were event free survival （EFS）, the rate of event free survival in SB-ALL group was 90.9% （30/33）, the rate of EFS in IB-ALL group was 83.3% （10/12） and the rate of EFS in HR-ALL was 27.3%（3/11）. The mean time of survival in SR-ALL group, MR-ALL group, and HR-ALL group were 75.2 m, 56.8 m, and 25.3 m, respectively. Two patients in HR-ALL group received HLA matched peripheral Blood stem cell transplantation and were event free survival for 5 years. [Conclusion] Chemotherapy according to clinical type could increase higher rate of event free survival in children ALL. But how to increase the rate of event free survival of HB-ALL should be studied. It might be a better method for HR-ALL children patients to choose hematopoitic stem cell transplantation if there were available donor.