Pichler Alexander S, Amador Catalina, Fujimoto Ayumi, Takeuchi Kengo, de Jong Daphne, Iqbal Javeed, Staber Philipp B
Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria.
Department of Pathology and Laboratory Medicine, University of Miami, Miami, Florida, USA.
Histopathology. 2025 Jan;86(1):119-133. doi: 10.1111/his.15376.
Peripheral T cell lymphomas (PTCLs) are a biologically diverse and aggressive group of non-Hodgkin lymphomas that originate from mature T cells, often presenting with complex clinical and morphological features. This review explores the challenges in diagnosing and classifying PTCLs, focusing on the intricate biology of the more common nodal entities. Advances in molecular diagnostics, such as mutational and gene expression profiling, have improved our understanding. However, the rarity and morphological variability of PTCLs continue to complicate the definition of biologically and clinically meaningful entities, as well as the application of current diagnoses in daily practice; these advancements have not yet translated into improved clinical outcomes. Standard therapies fail in most cases and lead to poor prognoses, highlighting the urgent need for improved therapeutic strategies. Precise characterisation of PTCL advances refined classification and supports the development of more targeted and effective treatments. Recent approaches have focused on biology-based risk stratification, either within specific entities or in an entity-agnostic manner. This development aims for improved treatment selection or even personalised treatment based on genetic, epigenetic and functional profiles.
外周T细胞淋巴瘤(PTCLs)是一组生物学特性多样且侵袭性强的非霍奇金淋巴瘤,起源于成熟T细胞,常表现出复杂的临床和形态学特征。本综述探讨了PTCLs诊断和分类方面的挑战,重点关注较为常见的淋巴结实体的复杂生物学特性。分子诊断技术的进展,如突变和基因表达谱分析,增进了我们的理解。然而,PTCLs的罕见性和形态学变异性继续使生物学和临床意义明确的实体的定义以及当前诊断在日常实践中的应用复杂化;这些进展尚未转化为改善的临床结果。大多数情况下标准疗法无效,预后不良,凸显了改进治疗策略的迫切需求。PTCLs的精确特征描述有助于完善分类,并支持开发更具针对性和有效性的治疗方法。最近的方法集中在基于生物学的风险分层上,无论是在特定实体内部还是以不考虑实体的方式。这一进展旨在根据遗传、表观遗传和功能特征改进治疗选择甚至实现个性化治疗。