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血管免疫母细胞性 T 细胞淋巴瘤:涵盖发病、病理、临床、治疗特征及近期进展的简要概述

英文原题:Angioimmunoblastic T-cell lymphoma: a concise overview encompassing the pathogenetic, pathological, clinical, therapeutical characteristics, and recent advances.

PubMed 2025/06/25(内容时间) Clin Exp Med Q2 · IF 4.5(JCR 2025)

研究概要

血管免疫母细胞性T细胞淋巴瘤(AITL)是一种罕见的外周T细胞淋巴瘤(PTCL)亚型,具有地域差异,起源于滤泡辅助性T(Tfh)细胞,以显著的免疫学参与为特征。

中文摘要

血管免疫母细胞性T细胞淋巴瘤(AITL)是外周T细胞淋巴瘤(PTCL)的一种罕见亚型,具有地域差异,起源于滤泡辅助性T(Tfh)细胞,以显著的免疫参与为特征。AITL的肿瘤微环境(TME)复杂,主要由T细胞、B细胞、浆细胞、滤泡树突状细胞和高内皮微静脉组成。在遗传学上,AITL表现为造血干细胞中TET2和DNMT3A突变的特点,而在Tfh细胞中可检测到RHOA和IDH2突变。随后,Tfh细胞开始释放多种趋化因子和细胞因子,调节与TME之间复杂的相互作用网络,促进AITL的发展。由于临床表现多样且实验室特征类似于多种良性疾病的变化,AITL的诊断仍然具有挑战性。目前已提出多种预测模型;然而,AITL的总体预后仍然较差。治疗策略应基于患者的年龄、身体状况和合并症。建议将参加临床试验作为初始治疗策略。自体干细胞移植(ASCT)用于AITL仍是一个持续争论的话题。针对复发/难治性(R/R)AITL已开展了大量多阶段临床试验。此外,CAR-T和CAR-NK治疗代表了值得进一步探索的AITL治疗前景广阔的途径。

展开英文摘要原文

Angioimmunoblastic T-cell lymphoma (AITL), a rare subtype of peripheral T-cell lymphoma (PTCL) with regional differences, originates from follicular T helper (Tfh) cells and is characterized by significant immunological involvement. The tumor microenvironment (TME) in AITL is complex, primarily composed of T cells, B cells, plasma cells, follicular dendritic cells and high endothelial venules. Genetically, AITL exhibits the characteristics of TET2 and DNMT3A mutations in hematopoietic stem cells, while RHOA and IDH2 mutations are detected in the Tfh cells. Subsequently, Tfh cells begin to release various chemokines and cytokines to regulate the intricate network of interactions with TME promoting development of AITL. Diagnosis remains challenging for AITL due to diverse clinical presentations and laboratory features resembling changes seen in multiple benign diseases. Several predictive models have been proposed; however, overall prognosis for AITL remains poor. Treatment strategies should be based on the patient's age, physical condition, and comorbidities. Participation in clinical trials is recommended as an initial treatment strategy. Autologous stem-cell transplantation (ASCT) for AITL still remains to be a subject of ongoing debate. Numerous multi-phase clinical trials have been carried out for relapsed/refractory (R/R) AITL. Moreover, CAR-T and CAR-NK therapy represents promising avenues that are worthy of further exploration for the treatment of AITL.

论文信息

作者
Feng Y、Ma Y、Li T、Liu M、Hong Z、Yin Q、Zheng M
第一作者单位
Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.China
通讯作者单位
Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. zmzk@sina.com.China
文献类型
综述
期刊
Clinical and experimental medicine2025 Jun 25
原文标识
PubMed 40560424 · DOI 10.1007/s10238-025-01754-4