Satou Akira, Takahara Taishi, Tsuzuki Toyonori
Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute 480-1195, Japan.
Diagnostics (Basel). 2022 Aug 18;12(8):2001. doi: 10.3390/diagnostics12082001.
Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells. PTCLs comprise numerous disease entities, with over 30 distinct entities listed in the latest WHO classification. They predominantly affect adults and elderly people and usually exhibit an aggressive clinical course with poor prognosis. According to their presentation, PTCLs can be divided into nodal, extranodal or cutaneous, and leukemic types. The most frequent primary sites of PTCLs are lymph nodes, with over half of cases showing nodal presentation. Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified. Adult T-cell leukemia/lymphoma also frequently affects lymph nodes. Recent pathological and molecular findings in nodal PTCLs have profoundly advanced the identification of tumor signatures and the refinement of the classification. Therefore, the therapies and pathological diagnosis of nodal PTCLs are continually evolving. This paper aims to provide a summary and update of the pathological and molecular features of nodal PTCLs, which will be helpful for diagnostic practice.
外周T细胞淋巴瘤（PTCL）是起源于成熟T细胞或NK细胞的罕见肿瘤。PTCL包含众多疾病实体，最新的世界卫生组织分类列出了30多种不同的实体。它们主要影响成年人和老年人，通常表现出侵袭性的临床病程，预后较差。根据其表现形式，PTCL可分为淋巴结型、结外型或皮肤型以及白血病型。PTCL最常见的原发部位是淋巴结，超过半数的病例表现为淋巴结受累。淋巴结型PTCL包括ALK阳性和ALK阴性间变性大细胞淋巴瘤；起源于T滤泡辅助细胞的淋巴结T细胞淋巴瘤；以及未另行特指的PTCL。成人T细胞白血病/淋巴瘤也常累及淋巴结。淋巴结型PTCL最近的病理和分子研究结果极大地推动了肿瘤特征的识别和分类的细化。因此，淋巴结型PTCL的治疗和病理诊断在不断发展。本文旨在总结和更新淋巴结型PTCL的病理和分子特征，这将有助于诊断实践。
