Department of Leukemia, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 428, Houston, TX, 77030, USA.
J Hematol Oncol. 2023 Mar 16;16(1):22. doi: 10.1186/s13045-023-01409-5.
Progress in the research and therapy of adult acute lymphoblastic leukemia (ALL) is accelerating. This analysis summarizes the data derived from the clinical trials conducted at MD Anderson between 1985 and 2022 across ALL subtypes. In Philadelphia chromosome-positive ALL, the addition of BCR::ABL1 tyrosine kinase inhibitors (TKIs) to intensive chemotherapy since 2000, improved outcomes. More recently, a chemotherapy-free regimen with blinatumomab and ponatinib resulted in a complete molecular remission rate of 85% and an estimated 3-year survival rate of 90%, potentially reducing the role of, and need for allogeneic stem cell transplantation (SCT) in remission. In younger patients with pre-B Philadelphia chromosome-negative ALL, the integration of blinatumomab and inotuzumab into the frontline therapy has improved the estimated 3-year survival rate to 85% across all risk categories. Our future strategy is to evaluate the early integration of both immunotherapy agents, inotuzumab and blinatumomab, with low-dose chemotherapy (dose-dense mini-Hyper-CVD-inotuzumab-blinatumomab) into the frontline setting followed by CAR T cells consolidation in high-risk patients, without any further maintenance therapy. In older patients, using less intensive chemotherapy (mini-Hyper-CVD) in combination with inotuzumab and blinatumomab has improved the 5-year survival rate to 50%. Among patients ≥ 65-70 years, the mortality in complete remission (CR) is still high and is multifactorial (old age, death in CR with infections, development of myelodysplastic syndrome or acute myeloid leukemia). A chemotherapy-free regimen with inotuzumab and blinatumomab is being investigated. The assessment of measurable residual disease (MRD) by next-generation sequencing (NGS) is superior to conventional assays, with early MRD negativity by NGS being associated with the best survival. We anticipate that the future therapy in B-ALL will involve less intensive and shorter chemotherapy regimens in combination with agents targeting CD19 (blinatumomab), CD20, and CD22 (inotuzumab). The optimal timing and use of CAR T cells therapy may be in the setting of minimal disease, and future trials will assess the role of CAR T cells as a consolidation among high-risk patients to replace allogeneic SCT. In summary, the management of ALL has witnessed significant progress during the past four decades. Novel combination regimens including newer-generation BCR::ABL1 TKIs and novel antibodies are questioning the need and duration of intensive chemotherapy and allogeneic SCT.
成人急性淋巴细胞白血病(ALL)的研究和治疗进展正在加速。本分析总结了 MD Anderson 自 1985 年至 2022 年在 ALL 各亚型临床试验中获得的数据。在费城染色体阳性 ALL 中,自 2000 年以来,将 BCR::ABL1 酪氨酸激酶抑制剂(TKI)加入强化化疗中,改善了预后。最近,无化疗方案采用blinatumomab 和 ponatinib,完全分子缓解率为 85%,估计 3 年生存率为 90%,可能降低缓解期同种异体干细胞移植(SCT)的作用和需求。在费城染色体阴性、年龄较小的前 B 型 ALL 患者中,将blinatumomab 和 inotuzumab 整合到一线治疗中,使所有风险类别的估计 3 年生存率提高到 85%。我们未来的策略是评估早期将两种免疫治疗药物——inotuzumab 和 blinatumomab——与低剂量化疗(密集 mini-Hyper-CVD-inotuzumab-blinatumomab)整合到一线治疗中,然后在高危患者中进行 CAR T 细胞巩固,无需任何进一步的维持治疗。在年龄较大的患者中,采用 mini-Hyper-CVD 联合 inotuzumab 和 blinatumomab 的化疗强度降低,5 年生存率提高至 50%。对于年龄≥65-70 岁的患者,完全缓解(CR)的死亡率仍然很高,且具有多因素性(年龄较大、CR 时感染死亡、骨髓增生异常综合征或急性髓系白血病的发展)。正在研究无化疗方案的 inotuzumab 和 blinatumomab。下一代测序(NGS)检测的可测量残留疾病(MRD)评估优于传统检测,NGS 早期的 MRD 阴性与最佳生存相关。我们预计,B-ALL 的未来治疗将涉及强度较低、时间较短的化疗方案,联合针对 CD19(blinatumomab)、CD20 和 CD22(inotuzumab)的药物。CAR T 细胞治疗的最佳时机和应用可能是在疾病最小化的情况下,未来的试验将评估 CAR T 细胞作为高危患者巩固治疗的作用,以替代同种异体 SCT。总之,过去四十年中 ALL 的治疗取得了重大进展。包括新一代 BCR::ABL1 TKI 和新型抗体在内的新型联合方案正在质疑强化化疗和同种异体 SCT 的必要性和持续时间。