t(8;21)急性髓性白血病特征和预后分析(英文)  

Characteristics and Prognostic Factors of Acute Myeloid Leukemia with t(8;21) (q22;q22)

作　　者：赖悦云[1] 邱镜滢[1] 江滨[1] 卢锡京[1] 黄晓军[1] 张艳[1] 刘艳荣[1] 史惠琳[1] 陆道培[1] 

机构地区：[1]北京大学人民医院血液病研究所,北京100044

出　　处：《中国实验血液学杂志》2005年第5期733-740,共8页Journal of Experimental Hematology

摘　　要：t(8;21)是急性髓性白血病最常见的染色体异常之一。t(8;21)AML具有独特的临床特征,化疗缓解率较高,生存期较长,但亦有预后较差的报道。为进一步探讨中国人群t(8;21)AML的临床特征及预后因素,对75例t(8;21)AML患者,其中包括68例FABM2,5例M4,2例M5进行了回顾性分析。结果表明:39例患者骨髓中可见Auer小体(52%),而骨髓嗜酸细胞增多(>5%)见于5例(6.7%);免疫分型高表达CD34和HLADR,仅有13例患者表达CD19(20.9%);细胞遗传学分析示62.5%患者具有附加染色体异常,主要附加异常类型为性染色体丢失(LOS),+4,del(9q)及+8;通过常规诱导化疗,完全缓解率82.7%;随访1-96月,19例复发,中位复发时间为10.5月(3-42月),中位生存时间是20个月,5年预期生存率为32.3%;多因素分析显示染色体核型,髓外白血病,年龄及缓解后治疗方式是影响生存的主要预后因素;伴有附加染色体异常患者的生存期较单纯t(8;21)患者短(P=0.019),而不同附加异常之间无明显差异;髓外白血病亦是不良的预后因素(P=0.012),年龄≤15岁患者比年龄>15岁者生存期长(P=0.045);接受造血干细胞移植的患者的预后好于单纯化疗者(P=0.030)。结论:中国人群t(8;21)AML具有不同于其他人群的特性,其预后相对较差,尤其是伴有附加染色体异常及髓外白血病者预后更差;对于具有不良预后因素的t(8;21)AML患者应该建议接受造血干细胞移植。The translocation t（8;21） （q22;q22） frequently associated with additional chromosomal aberrations is one of the most recurrent chromosomal abnormalities in AML. Clinically, this type of AML usually shows some specific characteristics and has a good response to chemotherapy with a high remission rate and a relatively long median survival.On the other hand, some reports also showed poor prognosis in AML patients with t（8;21 ） , and the associated bad prognosis factors have not been strongly established to date. To investigate this issue and to further identify the related characteristics of t（8 ;21 ） AML in China, 75 Chinese AML patients with t（8 ;21 ） were retrospectively analyzed. They comprised 68 cases of M2, five of M4 and two of M5 according to FAB classification. The results indicated that Auer rods were observed in 39 patients （52%） and marrow eosinophilia was detected in only 5 patients （6.7%）. These patients showed high level of HLA-DR and CD34 expression, while CD19 was detected in only 13 patients （20.9%）. Cytogenetically, 62.5 % cases had additional chromosomal abnormalities, and the main associated recurrent additional abnormalities were loss of a sex chromosome （LOS）, trisomy 4, del （9q） and trisomy 8. After conventional induction therapy, 62 patients attained complete remission （CR） resulting in the CR rate 82.7%. With a follow-up of 1 to 96 months, 19 cases relapsed at a median time of 10.5 months （range 3 to 42 months）. The median overall survival was 20 months, and the estimated 5-year overall survival （OS） rate was 32.3%. In multivariate analyses of prognostic factors,karyotype, extramedullary leukemia, age and post-remission therapy were of prognostic value for OS. Patients with additional chromosomal anomalies had shorter survival compared to those with t（8 ;21 ） only （P = 0.019）, no matter which kind of additional karyotype it was. Extramedullary leukemia was an adverse prognostic factor （ P = 0. 012）. Patients aged 15 years or l

关 键 词：t(8 21) 附加染色体异常 急性髓性白血病 

分 类 号：R733.7[医药卫生—肿瘤]