T细胞肿瘤是一类高度异质性的白血病和淋巴瘤，占所有淋巴组织恶性肿瘤的10-15％。由于罕见，传统上我们对T细胞白血病和淋巴瘤的理解落后于B细胞恶性肿瘤。然而，最近在基因表达和突变分析以及其他高通量方法方面，我们对T细胞分化的理解有了重大进展，从而更好地阐明了T细胞白血病和淋巴瘤的发病机制。在本综述中，我们概述了多种类型的T细胞白血病和淋巴瘤中发生的许多分子异常。这些知识的大部分已被用于改善第五版世界卫生组织的诊断标准。这些知识也正在用于改进预后，并确定新的治疗靶点。我们期望这种进展将继续，最终为T细胞白血病和淋巴瘤患者带来改善的预后。版权所有©2023 Australasia皇家病理学院。由Elsevier B.V.出版。保留所有权利。

T-cell neoplasms are a highly heterogeneous group of leukaemias and lymphomas that represent 10-15% of all lymphoid neoplasms. Traditionally, our understanding of T-cell leukaemias and lymphomas has lagged behind that of B-cell neoplasms, in part due to their rarity. However, recent advances in our understanding of T-cell differentiation, based on gene expression and mutation profiling and other high throughput methods, have better elucidated the pathogenetic mechanisms of T-cell leukaemias and lymphomas. In this review, we provide an overview of many of the molecular abnormalities that occur in various types of T-cell leukaemia and lymphoma. Much of this knowledge has been used to refine diagnostic criteria that has been included in the fifth edition of the World Health Organization. This knowledge is also being used to improve prognostication and identify novel therapeutic targets, and we expect this progress will continue, eventually resulting in improved outcomes for patients with T-cell leukaemias and lymphomas.Copyright © 2023 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.