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
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
作者信息
- 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
- 折叠
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.
Abstract
Objective To investigate the biological characteristics of biphenotypic acute leukemia (BAL) with t(8;21)(q22;q22) in children. Methods 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) without t(8 ;21) translocation detected during same period. Results The percentage of BAL with t (8 ;21) (q22;q22) in the 190 cases of the AML was 3.7% (7/190) . The BAL with t(8;21) of which the expression was t (8;21) (q22;q22) that the bone marrow cell morphology showed the AML -M2. The initial 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/ET0 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 unfavorable indicators of BAL may be concerned abnormal karyotypes, high expression of positivity of CD34. Combined 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Key words
children/acute myeloid leukemia/immunophenotype/t(8/21)/BAL引用本文复制引用
出版年
2012
NETL
NSTL
万方数据