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目的:探讨SIL-TAL1融合基因阳性儿童急性T淋巴细胞白血病(T-cell acute lymphoblastic leukemia,TALL)的临床特征、治疗方案及预后。方法:收集2005年4月至2012年11月间来我院治疗的101例T-ALL患儿的临床资料,比较SIL-TAL1阳性和SIL-TAL1阴性患儿的常见临床特征、早期治疗反应、微小残留病(minimal residual disease,M RD)、无事件生存率(event free survival,EFS)、无复发生存率(relapse free survival,RFS)及SIL-TAL1阳性T-ALL采用BCH-2003方案和CCLG-2008方案治疗的疗效。结果:在101例患儿中共22例携带SIL-TAL1融合基因(21.9%)。SIL-TAL1阳性(22例)和阴性患儿(79例)初诊时性别、年龄、对泼尼松反应及中枢神经系统累及均无明显差异,但SIL-TAL1阳性患儿初诊时外周血白细胞水平明显高于SIL-TAL1阴性患儿(P=0.009)。两组患儿在诱导缓解治疗后、延迟强化治疗Ⅱ前、维持治疗前的MRD水平无明显差异,但SIL-TAL1阳性患儿中巩固治疗前的MRD高水平者明显多于SIL-TAL1阴性患儿(P<0.05),延迟强化治疗I前的MRD高水平者似有增多的趋势(P>0.05)。两组患儿的5年EFS和RFS没有明显差异。将22例SIL-TAL1阳性患儿按CCLG-2008方案的分型标准重新划分危险度,BCH-2003组(10例)的危险度明显高于CCLG-2008组(12例,P=0.029),但两组患儿在常见临床特征、早期治疗反应、MRD水平、长期预后等方面均无明显差异。结论:SIL-TAL1阳性患儿初诊时外周血白细胞水平较高,临床疗效与SIL-TAL1阴性患儿没有显著差异。同时,SIL-TAL1阳性患儿对早期强化治疗可能反应性较差,BCH-2003方案可能更适于这一亚型的患儿。
Objective: To investigate the clinical features, treatment and prognosis of T-cell acute lymphoblastic leukemia (TALL) with SIL-TAL1 fusion gene in children. Methods: The clinical data of 101 children with T-ALL treated in our hospital from April 2005 to November 2012 were collected. The common clinical features of SIL-TAL1-positive and SIL-TAL1-negative children were compared. Early treatment response, Minimum residual disease (M RD), event free survival (EFS), relapse free survival (RFS), and SIL-TAL1-positive T-ALL were measured using the BCH-2003 protocol and CCLG -2008 treatment of the efficacy of the program. RESULTS: Twenty-two of the 101 children carried the SIL-TAL1 fusion gene (21.9%). There were no significant differences in gender, age, response to prednisone and central nervous system involvement between SIL-TAL1 positive (n = 22) and negative (n = 79) patients, but peripheral blood leukocyte levels Significantly higher than SIL-TAL1-negative children (P = 0.009). There was no significant difference in MRD levels between the two groups before induction of remission and delayed intensive treatment Ⅱ, but the level of MRD before consolidation therapy in SIL-TAL1-positive children was significantly higher than that in SIL-TAL1-negative children (P <0.05), there was a trend of increase of MRD before delayed intensive treatment of I (P> 0.05). There was no significant difference between the 5-year EFS and RFS in both groups. According to the classification standard of CCLG-2008, 22 cases of SIL-TAL1-positive children were re-divided into groups. The risk of BCH-2003 group was significantly higher than that of CCLG-2008 group (12 cases, P = 0.029) However, there was no significant difference between the two groups in terms of common clinical features, early treatment response, MRD level and long-term prognosis. Conclusion: The level of peripheral blood leukocytes in newly diagnosed SIL-TAL1-positive children is higher than that in SIL-TAL1-negative children. At the same time, children with SIL-TAL1-positive may not respond well to early intensive treatment, and the BCH-2003 regimen may be more suitable for this subtype of child.