Department of Hematology, The Second Xiangya Hospital, Central South University, Changsha, 410011, Hunan, China.
Hunan Key Laboratory of Tumor Models and Individualized Medicine, Changsha, 410011, Hunan, China.
Clin Exp Med. 2023 Dec;23(8):4219-4235. doi: 10.1007/s10238-023-01197-9. Epub 2023 Sep 27.
Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive subtype of peripheral T-cell lymphomas with its cell origin determined to be follicular helper T-cells. AITL is characterized by a prominent tumor microenvironment involving dysregulation of immune cells, signaling pathways, and extracellular matrix. Significant progress has been made in the molecular pathophysiology of AITL, including genetic mutations, immune metabolism, hematopoietic-derived microenvironment, and non-hematopoietic microenvironment cells. Early diagnosis, detection of severe complications, and timely effective treatment are crucial for managing AITL. Treatment typically involves various combination chemotherapies, but the prognosis is often poor, and relapsed and refractory AITL remains challenging, necessitating improved treatment strategies. Therefore, this article provides an overview of the pathogenesis and latest advances in the treatment of AITL, with a focus on potential therapeutic targets, novel treatment strategies, and emerging immunotherapeutic approaches.
血管免疫母细胞性 T 细胞淋巴瘤（AITL）是一种侵袭性外周 T 细胞淋巴瘤，其细胞起源被确定为滤泡辅助 T 细胞。AITL 的特征是存在明显的肿瘤微环境，涉及免疫细胞、信号通路和细胞外基质的失调。在 AITL 的分子病理生理学方面已经取得了重大进展，包括遗传突变、免疫代谢、造血衍生的微环境和非造血微环境细胞。早期诊断、严重并发症的检测以及及时有效的治疗对于 AITL 的管理至关重要。治疗通常涉及各种联合化疗，但预后通常较差，复发和难治性 AITL 仍然具有挑战性，需要改进治疗策略。因此，本文概述了 AITL 的发病机制和最新治疗进展，重点介绍了潜在的治疗靶点、新型治疗策略和新兴的免疫治疗方法。
