Mouse models are essential to study and comprehend normal and malignant hematopoiesis. The ideal preclinical model should mimic closely the human malignancy. This means that these mice should recapitulate the clinical behavior of the human diseases such as cancer and therapeutic responses with high reproducibility. In addition, the genetic mutational status, the cell phenotype, the microenvironment of the tumor and the time until tumor development occurs, should be mimicked in a preclinical model. This has been particularly challenging for human angioimmunoblastic lymphoma (AITL), one of the most prominent forms of peripheral T-cell lymphomas. A complex network of interactions between AITL tumor cells and the various cells of the tumor microenvironment has impeded the study of AITL pathogenesis in vitro. Very recently, new mouse models that recapitulate faithfully the major features of human AITL disease have been developed. Here, we provide a summary of the pathology, the transcriptional profile and genetic and immune-phenotypic features of human AITL. In addition, we give an overview of preclinical models that recapitulate more or less faithfully human AITL characteristics and pathology. These recently engineered mouse models were essential in the evaluation of novel therapeutic agents for possible treatment of AITL, a malignancy in urgent need of new treatment options.

小鼠模型对于研究和理解正常和恶性造血作用至关重要。理想的临床前模型应该密切模仿人类恶性肿瘤。这意味着这些小鼠应该以高重现性重现癌症等人类疾病的临床行为和治疗反应。此外，基因突变状态、细胞表型、肿瘤微环境以及肿瘤发生的时间，应该在临床前模型中进行模拟。这对于人类血管免疫母细胞淋巴瘤 (AITL) 来说尤其具有挑战性，AITL 是外周 T 细胞淋巴瘤最突出的形式之一。 AITL肿瘤细胞与肿瘤微环境的各种细胞之间复杂的相互作用网络阻碍了AITL发病机制的体外研究。最近，新的小鼠模型被开发出来，它忠实地概括了人类 AITL 疾病的主要特征。在这里，我们总结了人类 AITL 的病理学、转录谱以及遗传和免疫表型特征。此外，我们还概述了临床前模型，这些模型或多或少忠实地再现了人类 AITL 特征和病理学。这些最近设计的小鼠模型对于评估可能治疗 AITL 的新型治疗药物至关重要，AITL 是一种迫切需要新治疗方案的恶性肿瘤。