Departments of Oncology and Pathology, St. Jude Children's Research Hospital, Memphis, TN, 38105, USA.
Front Med. 2020 Dec;14(6):689-700. doi: 10.1007/s11684-020-0759-8. Epub 2020 Oct 19.
The cure rate of childhood acute lymphoblastic leukemia (ALL) has exceeded 90% in some contemporary clinical trials. However, the dose intensity of conventional chemotherapy has been pushed to its limit. Further improvement in outcome will need to rely more heavily on molecular therapeutic as well as immuno-and cellular-therapy approaches together with precise risk stratification. Children with ETV6-RUNX1 or hyperdiploid > 50 ALL who achieve negative minimal residual disease during early remission induction are suitable candidates for reduction in treatment. Patients with Philadelphia chromosome (Ph)-positive or Ph-like ALL with ABL-class fusion should be treated with dasatinib. BH3 profiling and other preclinical methods have identified several high-risk subtypes, such as hypodiplod, early T-cell precursor, immature T-cell, KMT2A-rearranged, Ph-positive and TCF-HLF-positive ALL, that may respond to BCL-2 inhibitor venetoclax. There are other fusions or mutations that may serve as putative targets, but effective targeted therapy has yet to be established. For other high-risk patients or poor early treatment responders who do not have targetable genetic lesions, current approaches that offer hope include blinatumomab, inotuzumab and CAR-T cell therapy for B-ALL, and daratumumab and nelarabine for T-ALL. With the expanding therapeutic armamentarium, we should start focus on rational combinations of targeted therapy with non-overlapping toxicities.
在一些当代临床试验中,儿童急性淋巴细胞白血病(ALL)的治愈率已经超过 90%。然而,传统化疗的剂量强度已经达到了极限。要进一步提高疗效,需要更加依赖分子治疗以及免疫和细胞治疗方法,并进行精确的风险分层。在早期缓解诱导期间达到微小残留病阴性的 ETV6-RUNX1 或超二倍体>50 的 ALL 患儿适合减少治疗。费城染色体(Ph)阳性或 Ph 样 ALL 伴 ABL 类融合的患者应接受达沙替尼治疗。BH3 分析和其他临床前方法已经确定了几种高危亚型,如低二倍体、早期 T 细胞前体、不成熟 T 细胞、KMT2A 重排、Ph 阳性和 TCF-HLF 阳性 ALL,这些亚型可能对 BCL-2 抑制剂 venetoclax 有反应。还有其他融合或突变可能作为潜在靶点,但尚未建立有效的靶向治疗。对于没有可靶向遗传病变的其他高危患者或早期治疗反应不佳的患者,目前有希望的方法包括针对 B-ALL 的blinatumomab、inotuzumab 和 CAR-T 细胞治疗,以及针对 T-ALL 的daratumumab 和 nelarabine。随着治疗手段的不断增加,我们应该开始关注靶向治疗与非重叠毒性的合理联合。