儿童急性髓系白血病细胞遗传学改变与预后分析

Analyses of karyotypic characteristics and prognosis in pediatric acute myeloblastic leukemia

目的探讨儿童急性髓系白血病（AML）细胞遗传学改变与预后的关系。方法根据细胞遗传学表现将128例初诊AML患儿分为4组：伴t（15；17）改变患儿为急性早幼粒细胞白血病（APL）组；伴t（8；21）／inv（16）（p13；q22）改变患儿为A组；正常核型及无其他组细胞遗传学改变患儿为B组；伴t（9；22）（q34；q11）或-7或复杂核型细胞遗传学改变患儿为C组。对各组患儿进行预后分析。结果128例AML患儿4年无事件生存率为（55．9±4．7）％，4年总生存率为（69．3±4．5）％；非APL患儿4年无事件生存率及总生存率分别为（49．9±5．2）％、（57．1±6．0）％。APL组、A组、B组及C组患儿的4年无事件生存率分别为（72．2±1．1）％、（66．3±7．7）％、（38．5±9．1）％、（20．1±12．3）％，差异均有统计学意义（P值均为0．000）；4年总生存率分别为（92．6±5．1）％、（69．4±7．9）％、（55．6±8．6）％、（30．0±12．3）％，差异均具有统计学意义（P值均为0．000）。结论细胞遗传学改变可作为儿童AML的独立预后因素，用于判断预后及疾病危险度分组，对AML患儿进行个体化治疗。

Objective Acute myeloblastic leukemia （AML） accounts for 15 to 25 percent of child- hood acute leukemias. Cytogenetic information is important for diagnosis, classification and prognosis of AML. Our aim was to analyze the relationship between kalyotypic characteristics and prognosis of childhood AML. Method According to karyotypic characteristics, 128 newly diagnosed children AML were separated into 4 subgroups： patients with t（15;17） （group APL）, patients with t（8;21 ）/inv（16） （group A）, pa- tients with - 7/t （9 ;22 ）/complex karyotypes （ group C） and the others （ group B）. Prognoses of these pa- tients were analyzed. Results The ages ranged from 1 to 16 years with the mean age of 7 years. 85 boys and 43 girls were included in this study. The 4-year event-free survival （EFS） and overall survival （OS） rates were （55.9 ±4.7）% and （69.3% ±4.5）%, respectively. The 4-year EFS and OS of non-M3-AML pa- tients were （49.9 ±5.2 ） % and （ 57.1 ±6.0 ） %, respectively. The probabilities of 4-year EFS of the four subgroups were （72.2±1. 1）%,（66.3±7.7）%,（38.5 ±9.1）% and （20.1 ±12.3）%, respectively （ P = 0. 000 ）. The probabilities of 4-year OS were （ 92.6±5.1 ） %, （ 69.4 ± 7.9 ） %, （ 55.6 ± 8.6 ） % and （ 30.0±12.3 ） %, respectively （ P = 0. 000 ）. Conclusion Cytogenetie aberrations seen in pediatrie AML had a significant impact on prognosis.