摘要
目的 对伴CBFB-MYH11融合基因阳性的初治的急性髓系白血病(acute myeloid leukemia, AML)患儿的临床特征进行分析。方法 对郑州大学附属儿童医院2017—2021年收治的初诊伴CBFB-MYH11融合基因阳性的急性髓系白血病患儿的临床特征进行回顾性分析。结果 142例AML患儿伴CBFB-MYH11融合基因阳性者9例(6.3%);中位发病年龄5岁(1~10岁);发病时中位白细胞数45.76×10^(9)/L[(9.12~111.29)×10^(9)/L];免疫分型主要表达CD13、CD33、CD34、CD117、HLA-DR;FAB分型,9例患儿中M4Eo 5例(55.6%),M2 2例(22.2%),M5 2例(22%)。有2例(22.2%)患儿伴KIT突变;经1个疗程诱导化疗完全缓解(Complete response, CR)率100%。结论 伴CBFB-MYH11患儿FAB分型主要以M4Eo为最常见,免疫分型主要以髓系幼稚粒细胞抗原及单核细胞抗原为主,伴CBFB-MYH11患儿诱导治疗缓解率高,治疗效果好,如巩固治疗后融合基因表达水平仍大于0.1%,可行异基因造血干细胞术。
Objective To analyze the clinical characteristics of newly diagnosed acute myeloid leukemia(AML) children with positive CBFB-MYH11 fusion gene.Methods The newly diagnosed children with CBFB-MYH11 enrolled in the Children’s Hospital of Zhengzhou University from 2017 to 2021.The clinical characteristics of children with fusion gene-positive acute myeloid leukemia were retrospectively analyzed.Results Nine cases(6.3%) of 142 AML children with positive CBFB-MYH11 fusion gene, with onset around the age of 5.The median number of white blood cells in the disease was 45.76×10^(9)/L and immunophenotype mainly expressed CD13, CD33, CD34, CD117, HLA-DR.Of these 9 patients, 5(55.6%) were M4 Eo, 2(22.2%) were M2 and 2(22.2%) were M5.In addition, there were 2 cases(22.2%) with KIT mutation, and these two patients underwent a course of chemotherapy in which the disease was completely relieved.Conclusion The FAB type of children with CBFB-MYH11 is mainly M4 Eo.The immunotype is mainly based on myeloid granulocyte antigen and monocyte antigen.Children with CBFB-MYH11 have a high remission rate and good treatment effect after induction therapy.In addition, the fusion gene expression level is still greater than 0.1% after consolidation therapy, allogeneic hematopoietic stem cell surgery can be used for treatment.
作者
马平
史利欢
田亮
周建文
毛彦娜
刘炜
MA Ping;SHI Li-huan;TIAN Liang;ZHOU Jian-wen;MAO Yan-na;LIU Wei(Deparment of Hematology and Oncology,Children's Hospital Affiliated to Zhengzhou University,Zhengzhou 450000,China)
出处
《医药论坛杂志》
2022年第19期15-18,共4页
Journal of Medical Forum
基金
河南省医学科技攻关计划联合共建项目(LHGJ20200626)。
