MDS和AML是增加发病率的克隆性造血干细胞疾病，其预后因共同发生的分子异常等因素而有所变化。这些髓系肿瘤中经常检测到TP53突变，并且预示着不良预后和已知的治疗抗性。本文及时评述了TP53改变的复杂性，并根据2022年国际共识分类（ICC）和世界卫生组织（WHO）新指南更新了诊断和预后。本文涉及最佳测试策略并审查当前和新兴的治疗方法。虽然这一分子亚组的治疗格局正在积极发展，但还需要进一步探索，以优化这一群体的未满足需求的护理。版权所有©2023 Elsevier Ltd。

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.Copyright © 2023 Elsevier Ltd. All rights reserved.