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目的探讨伴t(8;21)(q22;q22)易位的儿童急性双表型白血病(BAL)的生物学特征。方法分析7例伴t(8;21)(q22;q22)易位的儿童BAL,取同期诊断的30例t(8;21)阴性的急性髓细胞性白血病(AML)患儿作为对照组,分析其骨髓细胞形态学、细胞免疫学表型、细胞遗传学、分子生物学(MICM)特征。结果 7例伴t(8;21)(q22;q22)易位的儿童BAL占同期连续190例急性髓细胞性白血病(AML)的3.7%。骨髓细胞形态学均显示为AML-M2,分类中原始细胞均显著增多(均P<0.01);免疫表型均为髓细胞系伴B淋巴细胞系表达;CD34为高表达阳性;均有t(8;21)(q22;q22)染色体改变,且常伴有染色体复杂易位或缺失等改变;融合基因AML1/ETO检测均为阳性;7例患儿经治疗后完全缓解(CR)率71.4%,对兼顾AML和急性淋巴细胞白血病(ALL)的联合治疗方案效果较好。结论 BAL是急性白血病的一种亚型,其预后不良可能与染色体异常发生率高、CD34高表达阳性有关,对于BAL采用兼顾AML和ALL的联合治疗方案可提高其疗效。
Objective To investigate the biological characteristics of childhood acute double-leukemia (BAL) with t (8; 21) (q22; q22) translocations. Methods Totally 7 children with T (8; 21) (q22; q22) translocations who were translocated with t (8; 21) translocation were enrolled in this study. Twenty children with acute myeloid leukemia (AML) Analysis of its bone marrow cell morphology, cellular immunological phenotype, cytogenetics, molecular biology (MICM) features. Results Seven of the children with t (8; 21) (q22; q22) translocations accounted for 3.7% of the 190 consecutive cases of acute myeloid leukemia (AML) in the same period. The morphology of myeloid cells showed AML-M2, and the number of blasts in the samples were significantly increased (all P <0.01). The immunophenotypes were both myeloid cell lineage with B lymphocyte lineage, 8; 21) (q22; q22) chromosome changes, and often accompanied by complex chromosome translocation or deletion and other changes; fusion gene AML1 / ETO test were positive; 7 cases of children after treatment complete remission (CR) rate of 71.4% , The combination of AML and acute lymphoblastic leukemia (ALL) combined treatment works better. Conclusions BAL is a subtype of acute leukemia. The poor prognosis may be related to the high incidence of chromosomal abnormalities and the high expression of CD34. Combined treatment of BAL with AML and ALL may improve its efficacy.