Department of Medicine, Division of Hematology and Oncology, Georgetown University, 3800 Reservoir Road NW, Washington, D.C. 20007, USA.
Department of Medicine, Division of Hematology and Oncology, Georgetown University, 3800 Reservoir Road NW, Washington, D.C. 20007, USA; Department of Medicine, Division of Hematology-Oncology, University of Pennsylvania, 12 South Pavilion, 3400 Civic Center Boulevard, Philadelphia, PA 19104, USA.
Blood Rev. 2023 Jul;60:101055. doi: 10.1016/j.blre.2023.101055. Epub 2023 Feb 14.
MDS and AML are clonal hematopoietic stem cell disorders of increasing incidence, having a variable prognosis based, among others, on co-occurring molecular abnormalities. TP53 mutations are frequently detected in these myeloid neoplasms and portend a poor prognosis with known therapeutic resistance. This article provides a timely review of the complexity of TP53 alterations, providing updates in diagnosis and prognosis based on new 2022 International Consensus Classification (ICC) and World Health Organization (WHO) guidelines. The article addresses optimal testing strategies and reviews current and arising therapeutic approaches. While the treatment landscape for this molecular subgroup is under active development, further exploration is needed to optimize the care of this group of patients with unmet needs.
骨髓增生异常综合征和急性髓系白血病是克隆性造血干细胞疾病，发病率不断上升，其预后存在差异，部分取决于是否存在伴发的分子异常。TP53 突变在这些髓系肿瘤中经常被检测到，并预示着对已知治疗方法具有耐药性的不良预后。本文及时回顾了 TP53 改变的复杂性，根据新的 2022 年国际共识分类（ICC）和世界卫生组织（WHO）指南，提供了在诊断和预后方面的更新。文章介绍了最佳的检测策略，并回顾了当前和新兴的治疗方法。尽管这一分子亚群的治疗领域正在积极发展，但仍需要进一步探索，以优化这群具有未满足需求的患者的治疗。
