University of North Carolina, 170 Manning Drive, 1185A Physicians Office Building, CB # 7236, Chapel Hill, NC, 27599, USA.
Children's Hospital Los Angeles, 4650 Sunset Blvd, MS 54, Los Angeles, CA, 90027, USA.
Curr Oncol Rep. 2021 Feb 5;23(2):22. doi: 10.1007/s11912-020-01010-w.
Mixed phenotype acute leukemia (MPAL) is a rare subtype of acute leukemia with features of both acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). The review examines current definitions and controversies in classification of MPAL, new insights into genomic drivers and pathogenesis, recent evidence to support treatment recommendations, and opportunities for future research.
Recent collaborative efforts have made progress in understanding the genomic landscape and optimal therapy for MPAL. The preponderance of retrospective data supports beginning therapy with ALL directed regimens. Differences in prognosis for adult and children with MPAL have led to divergent approaches for therapy intensity, including use of stem cell transplantation consolidation. MPAL remains a challenging leukemia to understand, research, and treat due to low incidence, shifting and subjective approaches to classification, and innate biological heterogeneity. Ongoing research hopes to surmount these obstacles through prospective studies within large cooperative groups to provide new insight into targetable biology and further refine optimal therapy.
混合表型急性白血病(MPAL)是一种罕见的急性白血病亚型,具有急性淋巴细胞白血病(ALL)和急性髓细胞白血病(AML)的特征。本文综述了 MPAL 的分类定义和争议、基因组驱动因素和发病机制的新见解、支持治疗建议的最新证据,以及未来研究的机会。
最近的合作研究在理解 MPAL 的基因组景观和最佳治疗方法方面取得了进展。大量回顾性数据支持采用 ALL 定向方案开始治疗。成人和儿童 MPAL 之间预后的差异导致治疗强度的方法不同,包括使用干细胞移植巩固治疗。由于发病率低、分类方法不断变化且具有主观性,以及内在的生物学异质性,MPAL 仍然是一种难以理解、研究和治疗的白血病。正在进行的研究希望通过大型合作组的前瞻性研究来克服这些障碍,为可靶向的生物学提供新的见解,并进一步完善最佳治疗方法。