Warren Alpert Medical School of Brown University, Providence, RI, USA.
Division of Hematology and Medical Oncology, Weill Cornell Medicine, 1305 York Avenue, New York, NY, 10065, USA.
Curr Hematol Malig Rep. 2024 Jun;19(3):93-103. doi: 10.1007/s11899-024-00727-w. Epub 2024 Mar 7.
T-cell lymphomas (TCLs) are a group of rare subtypes of non-Hodgkin lymphoma derived from mature T-lymphocytes. Recent updates in lymphoma classification based on the cell-of-origin pathogenesis have shed new light on TCL epidemiology and outcomes. Contemporary regional consortia and international studies, including those conducted recently in Asia and South America, have provided an updated delineation of the major subtypes across various global regions.
Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), remains the most common subtype globally except in Asia, where extra-nodal NK-T cell lymphoma (ENKTL) has emerged as the most prevalent. Angioimmunoblastic T-cell lymphoma (AITL) is the second most common subtype globally except in South America where its incidence falls behind adult T-cell leukemia/lymphoma (ATLL) and ENKTL. ALK-negative anaplastic large cell lymphoma (ALCL) has been recognized as the second most common subtype in some parts of South America. Studies on the newly classified breast implant-associated ALCL (BIA-ALCL) are beginning to reveal its distribution and risk factors. Deciphering the epidemiology of TCLs is a challenging endeavor due to the rarity of these entities and ongoing refinement in classification. Collaborative efforts on prospective registries based on the most current WHO classifications will help capture the true epidemiology of TCL subtypes to better focus resources for diagnostic, prognostic, and therapeutic efforts.
T 细胞淋巴瘤（TCL）是一组源自成熟 T 淋巴细胞的罕见非霍奇金淋巴瘤亚型。基于起源细胞发病机制的淋巴瘤分类的最新更新，为 TCL 的流行病学和结果提供了新的视角。当代区域性联盟和国际研究，包括最近在亚洲和南美洲进行的研究，为各种全球区域的主要亚型提供了最新的描述。
除亚洲外，未另行说明的外周 T 细胞淋巴瘤（PTCL-NOS）仍然是最常见的全球亚型，在亚洲，结外 NK/T 细胞淋巴瘤（ENKTL）已成为最常见的亚型。血管免疫母细胞性 T 细胞淋巴瘤（AITL）是全球第二常见的亚型，仅次于南美洲，其发病率落后于成人 T 细胞白血病/淋巴瘤（ATLL）和 ENKTL。ALK 阴性间变性大细胞淋巴瘤（ALCL）已被认为是南美洲某些地区的第二大常见亚型。对新分类的乳房植入物相关 ALCL（BIA-ALCL）的研究开始揭示其分布和危险因素。由于这些实体的罕见性以及分类的不断完善，解析 TCL 的流行病学是一项具有挑战性的工作。基于最新的世界卫生组织分类的前瞻性登记处的合作努力将有助于捕捉 TCL 亚型的真实流行病学，以便更好地集中资源进行诊断、预后和治疗工作。
