48例急性混合细胞表型白血病患者的临床特征及预后

Clinical characteristics and prognosis of 48 patients with mixed phenotype acute leukemia

目的分析急性混合细胞表型白血病(MPAL)临床特点、生物学特征、疗效及预后。方法根据2008年WHO血液肿瘤分类标准,回顾性分析了48例M PAL患者(M PAL组)的病历资料,以同期50例伴淋系抗原表达的AML(Ly+AML)患者(Ly+AML组)作为对照。采用常规瑞士染色及细胞化学染色进行细胞形态学分析,采用流式细胞术进行细胞免疫分型,采用荧光原位杂交技术检测白血病常见融合基因,采用常规G显带技术分析染色体核型。结果 MPAL组发病中位年龄显著大于Ly+AML组(54岁vs 30岁,P<0.05),其CD34阳性率、异常染色体核型发生率及Ph染色体核型发生率也显著高于Ly+AML组(85.4%vs 50.0%,65.3%vs 35.7%,23.0%vs 0%,P均<0.05)。M PAL总体生存时间(OS)及无复发生存时间(RFS)均显著低于Ly+AM L组(P<0.05)。结论与Ly+AM L患者相比,M PAL患者的OS及RFS短,其预后不良可能与年龄、CD34高表达、Ph染色体阳性率高等因素有关。

Objective To investigate the clinical and biological characteristics and prognosis of mixed phenotype acute leukemia（ MPAL）. Methods Forty-eight patients diagnosed with MPAL（ MPAL group） by WHO 2008 criteria and50 patients diagnosed with Ly ＋ AML（ Ly ＋ AML group）,which is defined as the control group,in the same period were retrospectively analyzed. The cell morphology analysis was performed by swiss and cytochemical staining,the cell immunophenotype was detected by the flowcytometry,the fusion gene of leukemia was detected by fluorescence in situ hybridization,and the chromosome karyotype was analyzed by the standard technique of G-banding. Results The median age of MPAL group was larger than that of Ly ＋ AML group significantly（ 54 y vs 30 y,P〈0. 05）. The incidence of CD34 positive expression,bnormal karyotype and ph＋ in the MPAL group were significantly higher than those in the Ly ＋ AML group（ 85. 4% vs 50%,65. 3% vs 35. 7%,23% vs 0%,all P〈0. 05）. The OS and RFS of MPAL group were significantely lower than those of Ly ＋ AML group（ both P〈0. 05）. Conclusion Compared with Ly ＋AML group,MPAL group has lower OS and RFS; the poor prognosis of MPAL may due to the age,and high incidence of CD34 positive expression and ph＋.