伴t(8;21)(q22;q22)易位的儿童急性双表型白血病的生物学特征  

The biological characteristics of biphenotypic acute leukemia with t(8;21)(q22;q22) in children

作　　者：季正华[1] 计雪强[1] 黄益萍[1] 何亚香[1] 邵雪君[1] 徐俊[1] 胡绍燕[2] 王易[2] 何海龙[2] 赵文理[2] 

机构地区：[1]苏州大学附属儿童医院检验科,江苏苏州215003 [2]苏州大学附属儿童医院血液科,江苏苏州215003

出　　处：《苏州大学学报（医学版）》2012年第3期386-389,416,共5页Suzhou University Journal of Medical Science

摘　　要：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 （BAL） with t（ 8 ;21 ） （ q22 ; q22） in children. Methods characteristics of biphenotypic acute leukemia The characteristics of cellmorphology, immuno- phenotypes, chromosome karyotype and molecular biology of 7 cases of childhood BAL with t （ 8 ; 21 ） （q22;q22） were analyzed. The control group included 30 cases of acute myeloid leukemia（AML） with- out t（8 ;21 ） translocation detected during same period. Results The percentage of BAL with t （8;21） （q22 ;q22） in the 190 eases of the AML was 3.7% （7/190） . The BAL with t（8 ;21 ） of which the ex- pression was t （8 ;21 ）（q22;q22） that the bone marrow cell morphology showed the AML -M2. The ini- tial cells all increasd evidently in the classification（ all P 〈 0.01 ）. The immunophenotypes were all in the category of the expressions of the myeloid markers with B-lymphoid. CD34 had high expression of positivi- ty. There were changes of the chromosomal with t （8 ;21 ） （q22 ;q22）also with the complexly depletion or translocation. The detection results of the confluent genes AML1/ETO were positive. The total complete remission （CR） rate was 71.4%. It achieved good response to combined chemotherapy targeting to both myeloid and lymphocytic leukemia. Conclusion BAL is a rare type of acute leukemia. The unfavorableindicators of BAL may be concerned abnormal karyotypes, high expression of positivity of CD34. Com- bined chemotherapy targeting to both myeloid and lymphocytic leukemia contribute to improving survival.

关 键 词：儿童 急性髓细胞性白血病 免疫表型 t(8 21)易位 急性双表型白血病 

分 类 号：R733.71[医药卫生—肿瘤]