142例儿童急性髓系白血病初诊特征及诱导缓解疗效分析

Characteristics of 142 children with newly diagnosed acute myeloid leukemia and analysis of their induction outcome

目的分析儿童急性髓系白血病(AML)初诊临床及生物学特征,以期为儿童AML的进一步研究提供依据。方法回顾性分析142例2010年1月-2014年12月在广州市妇女儿童医疗中心确诊为AML患儿的初诊临床资料,并分析其与诱导缓解化疗疗效的关系。结果142例患儿男女比例1.45:1;中位年龄2.42(0.08~13.00)岁;临床表现主要为发热(80.30%)、髓外浸润(64.39%)及出血(46.97%);WBC中位计数为26.00(0.60~483.70)×10~9/L,Hb 71(31~124)g/L,PLT 38(1~404)×10~9/L;骨髓细胞形态前三位依次为M5(33.80%)、M2(25.35%)和M4(14.08%);免疫分型髓系抗原表达按比例依次为CD33、CD13和HLA-DR,伴有淋巴系抗原表达者占31.93%;异常核型检出率为53.66%;白血病相关融合基因检出率为33.33%;DAE及MAE方案诱导缓解化疗后总体完全缓解率(CR)为82.69%。结论142例AML患儿初诊临床特征与国内外文献报道存在不同程度的异同,提示深入开展多中心研究的必要性。

Objective To Analyze the clinical and biological characteristics of children with newly diagnosed acute myeloid leukemia（AML）, with a view to provide a basis for the further study of childhood AML. Methods Retrospectively analysed the clinical data of 142 children with newly diagnosed AML in Guangzhou Women and Children＇s Medical Center from January 2010 to December 2014, and its relationship with induction outcome. Results The male ： female sex ratio of 142 patients was 1.45 ： 1; with the median age of 2. 42（0. 08 ~13. 00） years; clinical manifestations were mainly fever（80. 30%）,extramedullary infiltration（64. 39%） and bleeding（46. 97%）; the median WBC was 26. 00（0. 60~483.70） ×10~9/L, Hb was 71（31~124） g/L,PLT was 38（1~404） ×10~9/L; the morphological type of bone marrow cells in top three were M5（ 33. 80%）, M2（25. 35%） and M4（ 14. 08%）; the expression of myeloid antigens in proportion respectively was CD33, CD13 and HLA-DR, and the expression of accompanied with lymphocytic antigen was 31.93 %; the detection rate of abnormal karyotype and leukemia-related fusion gene were 53. 66% and 33. 33% respectively; the overall CR rate was 82. 69%after induction chemotherapy with DAE and MAE. Conclusions The clinical characteristics of 142 children with newly diagnosed AML had a different degree of similarities and diversity compared to the literature reports from domestic and overseas, suggesting the further multicenter investigation is necessary.