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目的探讨影响儿童ALL疗效、长期生存与危险因素的关系。方法对采用德国儿童血液、肿瘤协作组ALL治疗方案(COALL-97/GPOH)治疗的50例ALL患儿的临床及实验室资料进行回顾性分析。结果 50例患儿全部完成诱导缓解治疗,完全缓解(CR)率100%。其中随访时间最长者103个月,最短6个月,中位数63个月,复发/死亡14例,无事件生存率(EFS)为64.3%。高危ALL(HR-ALL)19例,标危-ALL(LR-ALL)31例,EFS分别为66.3%和84.8%,其差异有统计学意义(χ2=2.783,P=0.010)。年龄≥10岁18例,<10岁32例,EFS分别为65.3%和82.8%,其差异有统计学意义(χ2=4.662,P<0.01)。初发时外周血白细胞计数≥25×109L-117例,<25×109L-133例,EFS分别为77.9%和82.8%,其差异有统计学意义(χ2=5.581,P<0.01)。B-ALL44例,T-ALL 6例,EFS分别为81.6%和53.3%,其差异有统计学意义(χ2=5.026,P<0.01)。细胞体外药物敏感试验及白血病细胞对泼尼松(PRED)、长春新碱(VCR)及门冬酰胺酶(ASP)的敏感程度积分(PVA-积分)和例数分别为3+4分24例、5-7分15例、8+9分11例,EFS分别为96.1%、79.7%和54.2%,其差异有统计学意义(χ2=3.737,P<0.002;χ2=2.448,P<0.028)。有完整染色体及基因检查资料的患儿27例。其中TEL/WT1(+)5例,死亡1例;BCR/ABL(+)4例,死亡3例;E2A/PBX1(+)并染色体t(1;19)和染色体t(3;7)t(12;22)各1例,2例均死亡。结论年龄、初发时外周血白细胞计数及免疫亚型是评估预后的重要因素;PVA-积分是对ALL临床危险因素分型的补充,使ALL临床分型更细致准确及治疗方案更加个体化。
Objective To explore the relationship between long-term survival and risk factors in children with ALL. Methods The clinical and laboratory data of 50 children with ALL treated with COALL-97 / GPOH in the German pediatric blood and cancer cohort were retrospectively analyzed. Results All the 50 children completed induction therapy and the complete remission (CR) rate was 100%. The longest follow-up was 103 months, the shortest 6 months, the median of 63 months, recurrence / death in 14 cases, event-free survival (EFS) was 64.3%. There were 31 cases of high risk ALL (HR-ALL) and 31 cases of standard risk-ALL (LR-ALL). The EFS were 66.3% and 84.8% respectively. The difference was statistically significant (χ2 = 2.783, P = 0.010). There were 18 cases aged ≥10 years and 32 cases <10 years old. The EFS were 65.3% and 82.8%, respectively. The difference was statistically significant (χ2 = 4.662, P <0.01). The number of peripheral blood leukocytes in the initial stage was ≥25 × 109L-117 cases, <25 × 109L-133 cases with EFS of 77.9% and 82.8%, respectively. The difference was statistically significant (χ2 = 5.581, P <0.01). B-ALL44 cases, T-ALL 6 cases, EFS were 81.6% and 53.3%, the difference was statistically significant (χ2 = 5.026, P <0.01). Sensitivity test of cells in vitro and leukemia cells’ sensitive degree integral (PVA-integral) to prednisone (PRED), vincristine (VCR) and asparaginase (ASP) and the number of cases were respectively 3 + 4 and 24 , 5 to 7 points in 15 cases and 8 + 9 points in 11 cases, the EFS were 96.1%, 79.7% and 54.2% respectively, with significant difference (χ2 = 3.737, P <0.002; χ2 = 2.448, P <0.028) . There are 27 cases of children with complete chromosome and genetic test data. There were 5 cases of TEL / WT1 (+) and 1 case of death; 4 cases of BCR / ABL (+) and 3 cases of death; E2A / PBX1 (+) and chromosome t (12; 22) in 1 case and 2 cases died. Conclusion Age, initial stage of peripheral blood leukocyte count and immune subtype are important factors to evaluate the prognosis. PVA-score is a supplement to the classification of clinical risk factors in ALL, which makes the clinical classification of ALL more detailed and accurate and the treatment plan more individualized.