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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 analyze the clinical features, biological characteristics, curative effect and prognosis of acute mixed cell phenotype leukemia (MPAL). Methods According to the WHO classification of blood cancer in 2008, 48 patients with M PAL (M PAL group) were retrospectively analyzed. The clinical data of 50 PAL patients with Lyme AML (Ly + AML) )as comparison. Cell morphology was analyzed by routine Swiss staining and cytochemical staining. Cell immunophenotyping was performed by flow cytometry. Common fusion genes of leukemia were detected by fluorescence in situ hybridization. Chromosome karyotypes were analyzed by conventional G - banding technique. Results The median age of MPAL group was significantly higher than that of Ly + AML group (54 vs. 30 years, P <0.05). The positive rate of CD34, the frequency of abnormal karyotype and the frequency of Ph chromosome were also significantly higher than those of Ly + AML (85.4% vs 50.0%, 65.3% vs 35.7%, 23.0% vs 0%, all P <0.05). M PAL overall survival time (OS) and recurrence-free survival time (RFS) were significantly lower than the Ly + AM L group (P <0.05). Conclusions Compared with patients with Ly + AM L, OS and RFS are short in patients with M PAL. The poor prognosis may be related to age, high expression of CD34 and high Ph chromosome positive rate.