t(8;21)急性髓系白血病的特征及预后分析

Analysis of characteristics and prognosis of acute myeloid leukemia with t(8;21)

目的探讨t(8;21)急性髓系白血病的特征及预后。方法回顾性分析80例初治t(8;21)AML患者,包括细胞遗传学G显带核型、免疫表型、细胞形态学、AML1/ETO融合基因、血象、乳酸脱氢酶(LDH)值及临床特征,并分析其预后因素。按染色体核型把患者分为单纯t(8;21)组(48例)、伴附加染色体异常组(32例)。结果80例t(8;21)AML患者中M277例,M41例,M4EO 2例;流式细胞仪检测t(8;21)AML患者免疫分型阳性率为:CD34 81.48%、CD13:96.29%、CD33:83.33%、HLA-DR90.74%、CD19:48.15%、CD56:55.55%;遗传学:48例(60.00%)为单纯t(8,21),32例(40.00%)有附加染色体异常,其中25例(31.25%)伴单纯性染色体丢失,7例(8.75%)为复杂变异型t(8;21)易位;初治时单纯t(8,21)组和伴有附加染色体组的年龄、性别、外周血和骨髓原始细胞数、血红蛋白(Hb)和血小板(PLT)计数、Auer小体、肝脾淋巴结浸润、LDH值、皮肤黏膜出血均无统计学意义(P均>0.05);伴有附加染色体组的外周血白细胞(WBC)计数和CD56阳性率均低于单纯组(P均<0.05)。伴附加染色体组与单纯组的CR率无明显差异(P>0.05);伴附加异常组的中位生存时间低于单纯t(8;21)组(P<0.05)。6例伴有髓外浸润患者的CR率和3年OS率均为0。结论伴附加染色体组与单纯t(8,21)组AML的临床特征有差异,附加染色体异常是t(8;21)AML的一种预后不良因素,伴有附加染色体异常的t(8;21)AML生存时间短于单纯t(8;21)者。

Objective To investigate the characteristics and prognosis of t（8 ;21 ） AML. Methods We retrospectively collected data of morphology, immunophenotype, karyotype, LDH changes, genetics, and clinical features from 80 cases of de novo AML with t（8 ;21 ） from January 2009 to June 2011, then we analysised the prognositc factors. These patients were divided into two groups： sole t（8 ;21） and t（8 ;21） with additional chromosomal abnormalities. Results Accord- ing to FAB classification,there were 77cases of M2,1 case of M4 ,2 cases of M4EO. The positive rate of stem/progenitor cell makers were： CD34 81.48% , CD13 ： 96. 29% , CD33 ： 83.33% , HLA - DR90. 74% , CD19 ： 48. 15% , CD56 ： 55.55%. Cytogenetically,32 cases（40% ） were accompanied by additional chromosomal abnormalities. There were no obvious differences between these two groups in their features of age distribution, WBC, HGB, PLT, bone marrow blast cells, Auer rods, eosinophilia, immunophenotype, hepatomegaly/splenomegaly/lymphadenectasis, LDH changes, as well as central nervous system leukemia occurrence and complete remisson rate of induction. But a lower initial WBC and a low- er positive rate of CD56 were observed in patients with additional chromosomal abnormalities. The median survival time ws much longer in patients with sole t（8 ;21 ）. CR rate and 3 - years - OS of patients with central nervous system leuke- mia were 0. Conclusion Additional chromosomal abnormality is an adverse factor for prognosis of t（8 ;21 ） AML. The median survival time of patients with additional aberrations was much shorter than that of those without.