伴CBFB-MYH11阳性的急性髓系白血病的临床分析  

Clinical Analysis of Acute Myeloid Leukemia with CBFB-MYH11-Positive

作　　者：曹婷婷[1] 周敏航[1] 袁磊[1] 王倩[1] 窦立萍[1] 徐媛媛[1] 王楠[1] 王莉莉[1] 于力[1] 靖彧[1] 

机构地区：[1]中国人民解放军总医院血液科,北京100853

出　　处：《中国实验血液学杂志》2013年第2期305-310,共6页Journal of Experimental Hematology

基　　金：解放军总医院苗圃基金(编号07MP03)

摘　　要：本研究旨在探讨伴CBFB-MYH11阳性的急性髓系白血病(acute myeloid leukemia,AML)的临床特征。回顾性分析293例AML中12例伴CBFB-MYH11阳性的急性髓系白血病患者的临床资料,包括性别、年龄、临床特点、免疫表型、治疗方案、疗效及预后。结果表明,在293例AML患者中伴CBFB-MYH11者占4%(12/293);本组12例发病中位年龄为32.5(21-57)岁;FAB分型为M4Eo 8例(66.7%),M4 4例(33.3%);初诊时中位白细胞数为19.8×109/L(2.46-164.3×109/L),WBC数的大于100×109/L者占16.7%(2/12);12例患者中,经1个疗程治疗达到完全缓解(CR)者10例(83.3%),经2个疗程治疗达CR者2例(16.7%),总CR率为100%;5年的RFS率和OS率分别为80%和83%。结论:伴CBFB-MYH11的患者多为FAB分型中的M4Eo或M4型,细胞多表达CD33、CD34、CD117、HLA-DR、CD15、CD64、CD14,伴CBFB-MYH11者诱导治疗完全缓解率高,总体生存时间及无复发生存时间长。The study was aimed to investigate the clinical characteristics of acute myeloid leukemia （AML） with CBFB-MYH11 gene. The clinical data of 12 cases were analyzed retrospectively, including age, clinical characteristics, immunophenotype, treatment protocols and efficacy as well as the prognosis. The results indicated that 12 patients with CBFB-MYH11 were detected in 293 AML patients. The median age of the 12 patients was 32.5（21 - 57） years otd. According to French- American- British （FAB） classification, 66.7% （8/12） patients was diagnosed as M4Eo and 33.3% patients was diagnosed as M4. At new diagnosis, the median WBC count was 19.8 ×10^9/L （2.46 - 164.30 ×10^9/L）. The WBC count 〉 100 ×10^9 was found in 16.7% patients （2/12）. The complete remission （CR） rate after 1 and 2 cycles of induction chemotherapy were 83. 3% and 16. 7% respectively, so the total CR rate was 100%. Estimated 5-year relapse-free survival （ RFS ） and overall survival （OS） were 80% and 83%, respectively. It is concluded that patients with CBFB-MYH11 are usually M4Eo and M4. Patients with this fusion gene are often associated with high frequency of CD33 ,CD34,CD117 ,HLA-DR, CD15 ,CD64 and CD14 expression. Patients with CBFB-MYH11 have a tendency of higher CR rate, longer RFS and OS.

关 键 词：急性髓系白血病 inv(16) CBFB—MYH11 化疗 造血干细胞移植 预后 

分 类 号：R733.71[医药卫生—肿瘤]