Sasaki Ko, Mitani Kinuko
Department of Hematology, Dokkyo Medical University School of Medicine.
Nihon Rinsho. 2009 Oct;67(10):1894-9.
Chronic myeloid leukemia (CML) is a paradigm for neoplasias that are defined by a unique genetic aberration, the BCR-ABL1 fusion gene. CML is also the best example for molecular target therapy. The development of protein tyrosine kinase inhibitor, imatinib, has entirely changed the strategy of therapy for CML. Nonetheless, many fields of pathogenesis for CML have not been elucidated, such as the mechanisms of blastic crisis, the causes of genetic instability including the inactivation of tumor suppressor genes, and oncogenic signaling pathways downstreams of the BCR-ABL1 fusion gene product. Herein, we review current knowledge on the molecular pathogenesis of CML.
慢性髓性白血病(CML)是由独特的基因畸变——BCR-ABL1融合基因所定义的肿瘤的范例。CML也是分子靶向治疗的最佳范例。蛋白酪氨酸激酶抑制剂伊马替尼的开发彻底改变了CML的治疗策略。尽管如此,CML发病机制的许多领域尚未阐明,如急变期的机制、包括肿瘤抑制基因失活在内的基因不稳定的原因,以及BCR-ABL1融合基因产物下游的致癌信号通路。在此,我们综述了关于CML分子发病机制的当前知识。