儿童急性髓系白血病138例临床分析  

Acute Myeloid Leukemia in Children:a Clinical Analysis of 138 Cases

作　　者：周丽红 陈启文[1] 黄正刚[1] 钟楠 

机构地区：[1]南昌大学第一附属医院儿科,南昌330006

出　　处：《实用临床医学（江西）》2017年第12期56-58,69,共4页Practical Clinical Medicine

摘　　要：目的了解急性髓系白血病(AML)的发病情况、疗效及预后。方法回顾性分析138例初发AML患儿的临床资料,并分析诱导化疗后骨髓完全缓解(CR)率及5年无病生存率与患儿性别、年龄、初发WBC计数及FAB分型的关系。结果 1)以男性和1~10岁患儿较多见;中位初诊年龄为8岁,其中有2例患儿初诊年龄<1岁。2)骨髓MICM分型检查:FAB分型中以M2亚型最多见;免疫分型除132例患儿单纯表达髓系分化抗原表达以外,4例同时伴有淋巴系抗原表达,2例为未表型;细胞遗传学中异常核型的检出率为77.8%,以复杂核型最多见;44例非急性早幼粒细胞白血病(APL)的AML患儿检测到ETO-AML1融合基因阳性,阳性率为45.4%,12例APL PML·RAR融合基因检测中10例阳性,检出率为83.3%。3)总诱导CR率为91.5%,5年无病生存率为66.7%。4)AML患儿诱导缓解化疗CR率与患儿性别、年龄、初发WBC计数及FAB分型无明显关系(均P>0.05),5年无病生存率与FAB分型有关(P<0.05)。结论儿童AML多发于1~10岁,总诱导CR率和5年无病生存率有待提高,对于部分疗效欠佳、预后不良的FAB分型的患儿,如何改变化疗方案或尽早采取移植以达到降低复发及病死率是改善儿童AML预后的关键。Objective To understand the pathogenesis,treatment and prognosis of acute myeloid leukemia(AML).Methods Clinical data of children with incipient AML were analyzed retrospectively.The relationships of complete remission(CR)and 5-year disease-free survival rates to the gender,age,white blood cell(WBC)count and FAB type were investigated after induction chemotherapy.Results The AML occurred most commonly in males and children aged 1-10 years.The median age was 8 years.Two cases were less than 1 year of age at first visit.MICM classification showed that M2 was the most prevalent FAB type.Among the 138 children,132 only expressed myeloid-specific antigens,4 expressed both myeloid and lymphocyte antigens,and 2 had no specific phenotypes.A total of 77.8% of these children had abnormal karyotypes and most of them exhibited complex karyotypes.ETO-AML1 gene fusion was detected in 44 children with non-acute promyelocytic leukemia(APL)(45.4%).The positive result was found in 10(83.3%)of 12 children who received APL-PML-RAR gene fusion examination.The total CR rate was91.5% and the 5-year disease-free survival rate was 66.7%.The CR rate was not correlated with the gender,age,WBC count and FAB type(P>0.05).However,the 5-year disease-free survival rate was associated with FAB type(P<0.05).Conclusion The AML occurs most frequently in children aged 1-10 years,and the CR rate and 5-year disease-free survival rate need to be improved.For AML children with poor outcome and prognosis of FAB classification,changing chemotherapy regimen and performing early transplantation are crucial for reducing recurrence and mortality and improving prognosis.

关 键 词：急性髓系白血病 儿童 疗效 预后 

分 类 号：R733.71[医药卫生—肿瘤]