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高度髓系分化和粒细胞增多与t(8;21) 冒烟型白血病相关。

High degree of myeloid differentiation and granulocytosis is associated with t(8;21) smoldering leukemia.

作者信息

Yamasaki H, Era T, Asou N, Sanada I, Matutes E, Yamaguchi K, Takatsuki K

机构信息

Second Department of Internal Medicine, Kumamoto University School of Medicine, Japan.

出版信息

Leukemia. 1995 Jul;9(7):1147-53.

PMID:7630188
Abstract

The t(8;21) is a frequent chromosome abnormality in acute myeloid leukemia (AML), particularly associated with M2 of the French-American-British (FAB) classification, but also found in a few patients with myelodysplastic syndrome (MDS). The two genes involved in the t(8;21) have been recently isolated and the cDNA of the AML1/ETO fusion gene identified. We have investigated a series of AML and MDS patients by a reverse transcriptase-polymerase chain reaction (RT-PCR) and analyzed the clinical and laboratory features of leukemia with t(8;21). The t(8;21) was only found in a subset of M2, which had the clinical and hematological features distinct from those M2 without t(8;21). M2 with t(8;21) was associated with a significantly higher myeloid differentiation and with a good response to chemotherapy. Moreover, among the patients with refractory anemia with excess of blasts in transformation (RAEB-T) the t(8;21) was also significantly associated with a higher myeloid differentiation and a good response to chemotherapy. M2 patients with t(8;21) could be distinguished on a number of hematological parameters, eg white blood cell count and percentage of bone marrow myeloblasts and promyelocytes, from RAEB-T carrying the t(8;21). Based on these findings we suggest that leukemia patients carrying t(8;21) can be grouped into two types; overt acute myeloid leukemia (M2) and smoldering or slowly evolving myeloid leukemia.

摘要

t(8;21)是急性髓系白血病(AML)中常见的染色体异常,尤其与法国-美国-英国(FAB)分类中的M2相关,但也在少数骨髓增生异常综合征(MDS)患者中发现。参与t(8;21)的两个基因最近已被分离出来,并且AML1/ETO融合基因的cDNA也已被鉴定。我们通过逆转录聚合酶链反应(RT-PCR)研究了一系列AML和MDS患者,并分析了伴有t(8;21)的白血病的临床和实验室特征。t(8;21)仅在M2的一个亚组中发现,该亚组具有与不伴有t(8;21)的M2不同的临床和血液学特征。伴有t(8;21)的M2与明显更高的髓系分化以及对化疗的良好反应相关。此外,在转化型原始细胞过多的难治性贫血(RAEB-T)患者中,t(8;21)也与更高的髓系分化和对化疗的良好反应显著相关。伴有t(8;21)的M2患者在一些血液学参数上,如白细胞计数、骨髓原始粒细胞和早幼粒细胞百分比等方面,可与携带t(8;21)的RAEB-T区分开来。基于这些发现,我们建议携带t(8;21)的白血病患者可分为两种类型:明显的急性髓系白血病(M2)和隐匿性或缓慢进展的髓系白血病。

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