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伴有t(8;21)(q22;q22)的B淋巴细胞和髓细胞系双表型急性白血病

B-Lymphoid and myeloid lineages biphenotypic acute leukemia with t(8;21)(q22;q22).

作者信息

He Guangsheng, Wu Depei, Sun Aining, Xue Yongquan, Jin Zhengming, Qiu Huiying, Tang Xiaowen, Miao Miao, Fu Zhengzheng, Ma Xiao, Wang Xiuli, Chen Zixin, Ruan Changgeng

机构信息

Jiangsu Insititute of Hematology, First Affiliated Hospital of Soochow University, Suzhou, 215006, Jiangsu, People's Republic of China.

出版信息

Int J Hematol. 2008 Mar;87(2):132-136. doi: 10.1007/s12185-008-0029-z. Epub 2008 Feb 22.

DOI:10.1007/s12185-008-0029-z
PMID:18288568
Abstract

By analyzing the characteristics of morphology, immune phenotype, chromosome karyotype and clinical manifestations of six cases of B-lymphoid and myeloid lineages biphenotypic acute leukemia (BAL) with t(8;21)(q22;q22), a new subgroup of BAL was reported. Bone marrow eosinophilia (more than 5%) and pseudo-Chediak abnormalities were not found. Auer rods were also not identified in four of six cases. Immunophenotype revealed B-lymphoid and myeloid lineages positive, together with frequent and high expression of CD34 and CD33, and weak expression of HLA-DR. In addition to t(8;21) chromosomal translocation, deletion of Y chromosome and complex chromosome abnormalities were also found. Chemotherapy for myeloid and lymphoid leukemia simultaneously produced good response in the patients. BAL with t(8; 21)(q22; q22) might be a new subgroup of BAL, and it was suggested that the leukemia clone with t(8;21)(q22;q22) might have originated from an early phase of hematopoiesis, and AML1/ETO fusion gene might be related to differentiation of B lymphocyte.

摘要

通过分析6例伴有t(8;21)(q22;q22)的B淋巴细胞系和髓细胞系双表型急性白血病(BAL)的形态学、免疫表型、染色体核型及临床表现特点,报道了BAL的一个新亚组。未发现骨髓嗜酸性粒细胞增多(超过5%)及假Chediak异常。6例中有4例未发现Auer小体。免疫表型显示B淋巴细胞系和髓细胞系均为阳性,同时CD34和CD33频繁高表达,HLA-DR弱表达。除t(8;21)染色体易位外,还发现Y染色体缺失及复杂的染色体异常。针对髓细胞系和淋巴细胞系白血病的化疗同时在患者中产生了良好反应。伴有t(8;21)(q22;q22)的BAL可能是BAL的一个新亚组,提示伴有t(8;21)(q22;q22)的白血病克隆可能起源于造血早期,且AML1/ETO融合基因可能与B淋巴细胞分化有关。

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本文引用的文献

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AML1-ETO needs a partner: new insights into the pathogenesis of t(8;21) leukemia.AML1-ETO需要一个伙伴:对t(8;21)白血病发病机制的新见解。
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Lineage-specific regulation of the murine RAG-2 promoter: GATA-3 in T cells and Pax-5 in B cells.小鼠RAG-2启动子的谱系特异性调控:T细胞中的GATA-3和B细胞中的Pax-5 。
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