Leukemia Section, Department of Medicine, Roswell Park Cancer Institute, Buffalo, New York 14263, USA.
Cancer. 2011 Apr 15;117(8):1583-94. doi: 10.1002/cncr.25690. Epub 2010 Nov 8.
The Philadelphia chromosome (Ph) is the most common cytogenetic abnormality associated with adult acute lymphoblastic leukemia (ALL). Before the advent of tyrosine kinase inhibitors (TKIs), Ph-positive ALL carried a dismal prognosis and was characterized by a poor response to most chemotherapy combinations, short remission durations, and poor survival rates. Outcomes for patients with Ph-positive ALL improved substantially with the introduction of TKIs, and the TKI imatinib induced complete remissions in >95% of patients with newly diagnosed Ph-positive ALL when it was combined with chemotherapy. However, imatinib resistance remains a problem in a substantial proportion of patients with Ph-positive ALL, and multiple molecular mechanisms that contribute to imatinib resistance have been identified. Second-generation TKIs (eg, dasatinib and nilotinib) have demonstrated promising efficacy in the treatment of imatinib-resistant, Ph-positive ALL. Future strategies for Ph-positive ALL include novel, molecularly targeted treatment modalities and further evaluations of TKIs in combination with established antileukemic agents. For this article, the authors reviewed past, current, and future treatment approaches for adult and elderly patients with Ph-positive ALL with a focus on TKIs and combined chemotherapeutic regimens.
费城染色体(Ph)是与成人急性淋巴细胞白血病(ALL)相关的最常见细胞遗传学异常。在酪氨酸激酶抑制剂(TKI)出现之前,Ph 阳性 ALL 预后不良,对大多数化疗联合的反应差,缓解持续时间短,生存率低。随着 TKI 的引入,Ph 阳性 ALL 患者的预后有了显著改善,当 TKI 伊马替尼与化疗联合使用时,可诱导新诊断的 Ph 阳性 ALL 患者中超过 95%的完全缓解。然而,伊马替尼耐药仍然是 Ph 阳性 ALL 患者中的一个问题,并且已经确定了多种导致伊马替尼耐药的分子机制。第二代 TKI(如达沙替尼和尼洛替尼)在治疗伊马替尼耐药的 Ph 阳性 ALL 中显示出良好的疗效。Ph 阳性 ALL 的未来治疗策略包括新型、分子靶向治疗方法,并进一步评估 TKI 与既定的抗白血病药物联合应用。在本文中,作者重点讨论了 TKI 和联合化疗方案,回顾了过去、现在和未来治疗 Ph 阳性 ALL 成人和老年患者的方法。