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关键词： 慢性粒细胞白血病(CML),cML急变,基因
慢性粒细胞白血病(CML)是一种起源于多能造血干细胞的血液系统恶性疾病。根据其临床进展，可分为慢性期(CP)，加速期(AP)和急变期(BC)。加速期和急变期表现为分化受阻，不受髓细胞生成调节因子的调节。研究CML急变的分子机制一直成为国内外的研究热点。近年来，许多临床和实验观察都显示CML急变时涉及到许多基因的改变，现将研究现状综述如下。
1 BCR-ABL基因与CML急变
早在1960年Nowell和Hungerford就在CML患者的白血病细胞中发现了费城染色体(Ph染色体)。1973年，Rowley等应用染色体分带技术，证明Ph染色体是由于t(9;22)(q34.1;q11.21)而形成的。
位于9q34上的ABL原癌基因易位至22q11的BCR基因3′端，形成BCR-ABL融合基因，其产物为相对分子质量为210×103的BCR-ABL融合蛋白(p210)。与正常的ABL蛋白相比，p210有更强的酪氨酸蛋白激酶活性，在体外能使造血祖细胞转化。Daley等［1］在骨髓移植模型中，将人BCR-ABL基因导入小鼠骨髓细胞，再输入接受致死剂量照射的同系小鼠，结果在绝大多数受体小鼠产生了类似人CML的病变，这些研究肯定了p210在白血病发生中起直接作用。
目录：
1 BCR-ABL基因与CML急变
2 其他癌基因与CML急变
3 抑癌基因与CML急变
4 其他基因与CML急变
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