Tang Jing-Yan, Xue Hui-Liang, Chen Jing, Pan Ci, Li Ben-Shang, Gu Long-Jun, Dong Lu, Hu Wen-Ting, Shen Shu-Hong, Zhou Min, Ye Qi-Dong, Jiang Hua, Luo Chang-Ying
Shanghai Children's Medical Center, School of Medicine, Shanghai Jiaotong University, Shanghai 200127, China.
Zhonghua Yi Xue Za Zhi. 2012 Feb 28;92(8):546-50.
To reduce the risk of therapy related complication during the treatment and keeps the long term event free survival, and to evaluate the results and risk factors of SCMC-lymphoblastic leukemia (ALL)-2005 protocol.
Designed the new protocol SCMC-ALL-2005 based on the previous protocol XH-99 for ALL. Divided the patients into low, median and high risk groups depends on risk factors including day 33 and 55 minimal residual disease (MRD) level. The higher risk group, the more intensive therapy was given. All the cases were registed on pediatric oncology network database (POND). All the abandonment patients were counted as event. From May 1(st) 2005 to April 30(th) 2009, 351 children who were newly diagnosed as B lineage ALL were enrolled in this study. The prognoses relating to risk grouping, age, mutation gene and MRD level were analyzed.
Up to June 30, 2011, 273 patients were followed up with median time 49 months (range 26 to 74 months). Three hundred and forty-five patients (98.29%) achieved complete remission on day 35 induction. 12 cases were younger than 1 year old (3.42%), 285 cases between 1 and 9 years old (81.20%), 54 cases 10 to 18 years old (15.38%). Five year event-free survival (EFS) was 34%, 72% and 63%, respectively. One hundred and fifty-six cases belonged to lowered risk (44.44%), 177 to middle risk (50.43%) and 18 to higher risk (5.13%). Five year EFS was 78%, 64% and 30%, respectively. In this study, 18 patients were detected positive for BCR/ABL, 3 for MLL/AF4, 16 for PBX/E2A, and 36 for TEL/AML. The 5 year EFS were 11%, 66%, 75% and 74%, respectively. A total of 300 cases were tested for MRD levels on day 35. Of them, 241 cases were with MRD ≤ 0.01% (negative), and 59 cases > 0.01% (positive). The 5 year relapse free survival (RFS) was 79% and 58%, respectively. Total 6 patients died of complication (1.71%). 18 patients were abundant treatment with no disease progress. 70 patients relapsed (19.94%), including 52 bone marrow, 8 central nerve system (CNS), 1 both in bone marrow and CNS, 1 second cancer (M(4)) and 8 testis. Five year overall survival (OS) and EFS are 84% and 69%.
The risk of therapy related death is low with the protocol SCMC-ALL-2005. MRD affects the prognosis. The long term prognosis is poor for high risk group, with BCR/ABL and positive MRD.
降低治疗期间治疗相关并发症的风险，维持长期无事件生存，并评估SCMC - 淋巴细胞白血病（ALL）-2005方案的治疗结果及危险因素。
基于之前的ALL方案XH - 99设计新的SCMC - ALL - 2005方案。根据包括第33天和第55天微小残留病（MRD）水平等危险因素将患者分为低、中、高风险组。风险组越高，给予的治疗越强化。所有病例均登记于儿科肿瘤网络数据库（POND）。所有放弃治疗的患者均计为发生事件。2005年5月1日至2009年4月30日，351例新诊断为B系ALL的儿童纳入本研究。分析了与风险分组、年龄、突变基因和MRD水平相关的预后情况。
截至2011年6月30日，273例患者接受随访，中位随访时间49个月（范围26至74个月）。345例患者（98.29%）在诱导治疗第35天达到完全缓解。12例患者年龄小于1岁（3.42%），285例患者年龄在1至9岁之间（81.20%），54例患者年龄在10至18岁之间（15.38%）。5年无事件生存（EFS）率分别为34%、72%和63%。156例属于低风险组（44.44%），177例属于中风险组（50.43%），18例属于高风险组（5.13%）。5年EFS率分别为78%、64%和30%。本研究中，18例患者检测出BCR/ABL阳性，3例MLL/AF4阳性，16例PBX/E2A阳性，36例TEL/AML阳性。5年EFS率分别为11%、66%、75%和74%。共300例患者在第35天检测MRD水平。其中，241例患者MRD≤0.01%（阴性），59例患者MRD>0.01%（阳性）。5年无复发生存（RFS）率分别为79%和58%。共有6例患者死于并发症（1.71%）。18例患者接受过度治疗但无疾病进展。70例患者复发（19.94%），包括52例骨髓复发，8例中枢神经系统（CNS）复发，1例骨髓和CNS均复发，1例继发性癌症（M(4)），8例睾丸复发。5年总生存（OS）率和EFS率分别为84%和69%。
SCMC - ALL - 2005方案治疗相关死亡风险较低。MRD影响预后。高风险组、伴有BCR/ABL及MRD阳性患者的长期预后较差。
