结节外围T细胞淋巴瘤的病理生物学:当前的理解和未来的方向
Bettina Bisig1, Kerry J Savage2, Laurence De Leval3
1Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Lausanne.
Haematologica
|December 1, 2023
概括
周围T细胞淋巴瘤 (PTCL) 分类的近期进展突显了ALK阴性ALCL的遗传异质性以及TFHL和PTCL,NOS亚组的细胞起源的重要性.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 免疫学 免疫学 免疫学
背景情况:
- 周围T (和NK) 细胞淋巴瘤 (PTCL) 主要以结节疾病的形式存在.
- PTCL包括形大细胞淋巴瘤 (ALCL),毛囊辅助T细胞淋巴瘤 (TFHL) 和PTCL,没有另有说明 (NOS).
- 最近的基因组和分子洞察力导致了2022年淋巴瘤分类的重大更新.
研究的目的:
- 审查有关节点PTCL实体病理学和生物学现有知识.
- 强调最近的发现和PTCL的潜在瘤机制.
- 讨论新分类对理解PTCL异质性的影响.
主要方法:
- 对PTCL最近的基因组和分子特征研究进行了审查.
- 分析了2022年更新的淋巴瘤分类.
- 结节PTCL病理和生物特征数据的综合.
主要成果:
- 负ALK的ALCL表现出遗传异质性,在20-30%的病例中出现DUSP22重组.
- TFHL分类受到细胞起源的影响,共享致癌途径和表观遗传突变.
- 新出现的数据表明,基于免疫特征的PTCL,NOS亚组的细胞起源相关性.
- 艾普斯坦-巴尔病毒阳性结节T/NK细胞淋巴瘤现在由于攻击性行为而被分别分类.
结论:
- 节点PTCL分类随着对病理生物学的更深入理解而不断发展.
- 遗传异质性和细胞起源是分类PTCL亚型的关键决定因素.
- 未来的研究应该专注于改进PTCL分类,并根据这些见解确定新的治疗点.
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