Division of Hematology and Oncology, Department of Medicine, University of Virginia Cancer Center, Charlottesville, VA.
Department of Pathology, University of Virginia Cancer Center, Charlottesville, VA.
Blood. 2024 Oct 31;144(18):1898-1909. doi: 10.1182/blood.2023021788.
Rare subtypes of peripheral T-cell lymphoma (PTCL) including enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and hepatosplenic T-cell lymphoma (HSTCL) are underrepresented in most registries and clinical studies. Most of the literature is obtained from small case series, single-institution retrospective studies, and subgroup analyses of the largest studies with few recent and ongoing exceptions. Although the pathogenesis and biology of these entities have yet to be fully elucidated, global efforts by the scientific community have started to shed some light on the most frequently deregulated pathways. In this review, we highlight the most pertinent clinical and pathologic features of rare subtypes of PTCL including EATL/MEITL, SPTCL, and HSTCL. We also summarize the results of recent developments identifying potential targets for novel therapeutic strategies based on molecular studies. Finally, we highlight the underrepresentation of these rare subtypes in most clinical trials, making evidence-based therapeutic decisions extremely challenging.
罕见的外周 T 细胞淋巴瘤（PTCL）亚型，包括肠病相关 T 细胞淋巴瘤（EATL）、单一表型上皮嗜性肠 T 细胞淋巴瘤（MEITL）、皮下脂膜炎样 T 细胞淋巴瘤（SPTCL）和肝脾 T 细胞淋巴瘤（HSTCL），在大多数登记处和临床研究中代表性不足。大多数文献来自小病例系列、单一机构回顾性研究，以及最大研究的亚组分析，只有少数是最近和正在进行的研究。尽管这些实体的发病机制和生物学尚未完全阐明，但科学界的全球努力已经开始揭示最常失调的途径。在这篇综述中，我们重点介绍了罕见的 PTCL 亚型的最相关的临床和病理特征，包括 EATL/MEITL、SPTCL 和 HSTCL。我们还总结了最近的分子研究确定新型治疗策略潜在靶点的结果。最后，我们强调了这些罕见亚型在大多数临床试验中的代表性不足，使得基于证据的治疗决策极具挑战性。
