Fukumoto Kota
Department of Hematology, Ageo Central General Hospital.
Rinsho Ketsueki. 2022;63(12):1657-1667. doi: 10.11406/rinketsu.63.1657.
Peripheral T-cell lymphomas (PTCLs) are mature T-cell and natural killer (NK) cell neoplasms with poor prognosis and no available standard treatment. The 2016 revision of the World Health Organization classification of hematological malignancies recognized a new group of nodal T-cell lymphoma with T follicular helper cell phenotype due to the presence of a group of PTCLs, not otherwise specified. This new subgroup of tumors showed clinical and molecular similarities with angioimmunoblastic T-cell lymphoma (AITL). AITL patients have mutations in the Vav guanine nucleotide exchange factor-1 (VAV1) gene. We established a transgenic mouse model harboring VAV1 gene mutation. These mice developed tumors that mimic human T-lymphoblastic lymphoma and the newly proposed PTCL-GATA3 subtype. To develop personalized treatment strategies by analyzing subgroup-specific mouse models, our study supports the establishment of PTCL subgroups based on genetic alterations or gene expression profiles.
外周T细胞淋巴瘤（PTCLs）是成熟T细胞和自然杀伤（NK）细胞肿瘤，预后较差且尚无可用的标准治疗方法。2016年世界卫生组织血液系统恶性肿瘤分类修订版因存在一组未另行规定的PTCLs，确认了一组具有T滤泡辅助细胞表型的新的结内T细胞淋巴瘤。这一新的肿瘤亚组在临床和分子方面与血管免疫母细胞性T细胞淋巴瘤（AITL）相似。AITL患者的Vav鸟嘌呤核苷酸交换因子-1（VAV1）基因存在突变。我们建立了携带VAV1基因突变的转基因小鼠模型。这些小鼠发生的肿瘤类似于人类T淋巴母细胞淋巴瘤和新提出的PTCL-GATA3亚型。为了通过分析亚组特异性小鼠模型制定个性化治疗策略，我们的研究支持基于基因改变或基因表达谱建立PTCL亚组。
