1236例儿童急性白血病住院病例分析

Large Sample Clinical Analysis of Patients with Children Acute Leukemia in Single Center

为了研究儿童急性白血病的流行病学,对2004年4月至2010年4月在中国医学科学院血液病医院住院的1236例儿童急性白血病患儿发病情况进行回顾性调查,分析住院患者中儿童急性白血病的发病年龄、发病时间、危险因素、各亚型分布以及分子流行病学等特征。结果表明:ALL、AML患儿男女比例为分别为1.80∶1及1.73∶1。发病年龄高峰为2岁至6岁,中位年龄6岁,其中ALL发病年龄高峰为2-5岁,AML没有明显的发病年龄高峰。冬季为患儿发病及出生季节高峰,1月份为患儿发病及出生月份高峰。免疫学分型显示,631例ALL患者中B-ALL占89%,T-ALL占9%。在361例AML中M0-M7各亚型白血病所占比例分别为0.3%、2.2%、29.8%、20.9%、8.1%、25.2%、4.1%、4.6%。进行分子生物学检测表明,631例ALL儿童中TEL/AML1阳性占23%,BCR/ABL阳性占7.4%,MLL阳性占4.1%,E2A/PBX1阳性占2.1%。在361例AML儿童中,19%患儿AML1/ETO融合基因阳性,18%PML/RARα融合基因阳性,4.2%CBFβ/MYH11融合基因阳性。结论 :不同性别、年龄、季节、环境及遗传背景对儿童急性白血病发病有影响,白血病各亚型发病率不一。

In order to investigate the epidemiology of childhood acute leukemia （ CAL）, such as onset age and time, risk factor, subtypes distribution and genetics, 1236 CAL patients admitted in blood disease hospital of Chinese Academy of Medical Sciences for treatment from April 2004 to April 2010 were analyzed retrospectively. The results showed that the sex ratio of ALL and AML patients were 1.80：1 and 1.73：1 respectively; the average peak age of incidence lasted from 2 to 6 years with the median age of 6 years, while the ALL peak age of incidence lasted from 2 to 5 years but AML showed no significant peak age of incidence. Winter, especially January was the peak time for both onset and birth. Among all the 631 ALL patients who had already been immunophenotyped, B-ALL patients accounted for 83%, T-ALL patients accounted for 9%. Among 361 AML patients, sub-leukemia phenotype from M0 to M7 accoumed for 0.3%, 2. 2%, 29.8%, 20.9%, 8.1%, 25.2%, 4.1% and 4.6% respectively. Among 631 pediatric ALL patients who had been examined by using molecular biology technique, the positive rate of TEL/AML1 , BCR/ABL, MLL and E2A/ PBX1 were 23%, 7.4%, 4.1%, 2.1% respectively. Among 361 pediatric AML patients who had been examined by using molecular biology technique, 19% of the patients showed positive AML1/ETO fusion gene, 18% of the patients showed positive PML/RARot fusion gene, while 4.2% of patients showed positive CBFfl/MYH11. It is concluded that the onset of pediatric aute leukemia is influenced by age, season, environment and different genetic background.