Division of Hematology and Oncology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI 48109-5936, USA.
Leukemia. 2011 Jan;25(1):7-22. doi: 10.1038/leu.2010.238. Epub 2010 Nov 19.
Although only 5000 new cases of chronic myeloid leukemia (CML) were seen in the United States in 2009, this neoplasm continues to make scientific headlines year-after-year. Advances in understanding the molecular pathogenesis coupled with exciting developments in both drug design and development, targeting the initiating tyrosine kinase, have kept CML in the scientific limelight for more than a decade. Indeed, imatinib, a small-molecule inhibitor of the leukemia-initiating Bcr-Abl tyrosine kinase, has quickly become the therapeutic standard for newly diagnosed chronic phase-CML (CP-CML) patients. Yet, nearly one-third of patients will still have an inferior response to imatinib, either failing to respond to primary therapy or demonstrating progression after an initial response. Significant efforts geared toward understanding the molecular mechanisms of imatinib resistance have yielded valuable insights into the cellular biology of drug trafficking, enzyme structure and function, and the rational design of novel small molecule enzyme inhibitors. Indeed, new classes of kinase inhibitors have recently been investigated in imatinib-resistant CML. Understanding the pathogenesis of tyrosine kinase inhibitor resistance and the molecular rationale for the development of second and now third generation therapies for patients with CML will be keys to further disease control over the next 10 years.
尽管 2009 年在美国仅观察到 5000 例新的慢性髓性白血病 (CML) 病例,但这种肿瘤仍年复一年地成为科学头条新闻。对分子发病机制的理解的进步,加上针对起始酪氨酸激酶的药物设计和开发方面的令人兴奋的发展,使 CML 在科学领域中保持了十多年的关注。实际上,伊马替尼是白血病起始 Bcr-Abl 酪氨酸激酶的小分子抑制剂,已迅速成为新诊断的慢性期 CML (CP-CML) 患者的治疗标准。然而,仍有近三分之一的患者对伊马替尼的反应较差,要么对初始治疗无反应,要么在初始反应后出现进展。针对了解伊马替尼耐药的分子机制的大量努力为药物转运、酶结构和功能的细胞生物学以及新型小分子酶抑制剂的合理设计提供了有价值的见解。实际上,最近已经在伊马替尼耐药性 CML 中研究了新的激酶抑制剂类别。了解酪氨酸激酶抑制剂耐药的发病机制以及为 CML 患者开发第二代和第三代治疗方法的分子原理将是在未来 10 年内进一步控制疾病的关键。