Engen Caroline Benedicte Nitter, Wergeland Line, Skavland Jørn, Gjertsen Bjørn Tore
Center for Cancer Biomarkers CCBIO, Department of Clinical Science, University of Bergen, Bergen N-5020, Norway.
Department of Internal Medicine, Hematology Section, Haukeland University Hospital, Bergen N-5021, Norway.
J Clin Med. 2014 Dec 15;3(4):1466-89. doi: 10.3390/jcm3041466.
Internal tandem duplications (ITDs) of the gene encoding the Fms-Like Tyrosine kinase-3 (FLT3) receptor are present in approximately 25% of patients with acute myeloid leukemia (AML). The mutation is associated with poor prognosis, and the aberrant protein product has been hypothesized as an attractive therapeutic target. Various tyrosine kinase inhibitors (TKIs) have been developed targeting FLT3, but in spite of initial optimism the first generation TKIs tested in clinical studies generally induce only partial and transient hematological responses. The limited treatment efficacy generally observed may be explained by numerous factors; extensively pretreated and high risk cohorts, suboptimal pharmacodynamic and pharmacokinetic properties of the compounds, acquired TKI resistance, or the possible fact that inhibition of mutated FLT3 alone is not sufficient to avoid disease progression. The second-generation agent quizartinb is showing promising outcomes and seems better tolerated and with less toxic effects than traditional chemotherapeutic agents. Therefore, new generations of TKIs might be feasible for use in combination therapy or in a salvage setting in selected patients. Here, we sum up experiences so far, and we discuss the future outlook of targeting dysregulated FLT3 signaling in the treatment of AML.
编码Fms样酪氨酸激酶3(FLT3)受体的基因内部串联重复(ITD)存在于约25%的急性髓系白血病(AML)患者中。该突变与预后不良相关,异常的蛋白质产物被认为是一个有吸引力的治疗靶点。已经开发了多种靶向FLT3的酪氨酸激酶抑制剂(TKI),但尽管最初抱有乐观态度,但在临床研究中测试的第一代TKI通常仅诱导部分和短暂的血液学反应。普遍观察到的有限治疗效果可能由多种因素解释;广泛预处理和高风险队列、化合物欠佳的药效学和药代动力学特性、获得性TKI耐药性,或者单独抑制突变的FLT3不足以避免疾病进展这一可能事实。第二代药物quizartinb显示出有前景的结果,并且似乎比传统化疗药物耐受性更好、毒性更小。因此,新一代TKI可能可用于联合治疗或在选定患者的挽救治疗中使用。在此,我们总结迄今的经验,并讨论在AML治疗中靶向失调的FLT3信号传导的未来前景。