慢性髓性白血病

Letícia Antunes Muniz Ferreira
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引用次数: 1

摘要

慢性粒细胞性白血病(CML)是一种慢性克隆性骨髓增生性疾病,其特征是左侧白细胞增多、脾肿大和费城(Ph)染色体的存在,这是由于染色体9q34和22q11长臂之间的相互平衡易位,产生具有酪氨酸激酶活性增加的杂交蛋白BCR-ABL。BCR-ABL蛋白存在于所有CML患者中,其过度活跃会触发细胞增殖效应物和细胞凋亡抑制剂的释放,其活性是CML最初致癌的原因。本章将从CML的发现、疾病进展的分子和表观遗传学机制到目前的治疗方法进行综述。
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Chronic Myeloid Leukemia
Chronic myelogenous leukemia (CML) is a chronic clonal myeloproliferative disease characterized by left leukocytosis, splenomegaly, and the presence of the Philadelphia (Ph) chromosome, which results from the reciprocal and balanced translocation between the long arms of chromosomes 9q34 and 22q11, generating the hybrid protein BCR-ABL, with increased tyrosine kinase activity. The BCR-ABL protein is present in all patients with CML, and its hyperactivity triggers the release of effectors of cell proliferation and inhibitors of apoptosis, and its activity is responsible for the initial oncogenesis of CML. This chapter will review CML from its discovery, molecular and epigenetic mechanisms of disease progression to current treatments.
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