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结节性外周 T 细胞淋巴瘤的病理学和分子特征

英文原题:Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas.

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Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas.

PubMed 2022/08/18(内容时间) Diagnostics (Basel) Q1 · IF 3.8(JCR 2025)

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中文摘要

外周T细胞淋巴瘤(PTCLs)是一类不常见的起源于成熟T细胞或NK细胞的肿瘤。PTCLs包括众多疾病实体,最新WHO分类中列出了30多种不同的实体。它们主要影响成人和老年人,通常表现出侵袭性临床病程且预后不良。根据其表现,PTCLs可分为结内型、结外型或皮肤型以及白血病型。PTCLs最常见的原发部位是淋巴结,超过半数病例表现为结内受累。结内PTCLs包括ALK阳性和ALK阴性间变性大细胞淋巴瘤;伴滤泡辅助性T细胞起源的结内T细胞淋巴瘤;以及PTCL,非特指型。成人T细胞白血病/淋巴瘤也常累及淋巴结。近年来结内PTCLs的病理学和分子学发现极大地推进了肿瘤特征的识别和分类的完善。因此,结内PTCLs的治疗和病理诊断在不断演变。本文旨在总结和更新结内PTCLs的病理学和分子学特征,这将有助于诊断实践。

展开英文摘要原文

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.

论文信息

作者
Satou A、Takahara T、Tsuzuki T
单位
Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute 480-1195, Japan.Japan
文献类型
综述
期刊
Diagnostics (Basel, Switzerland)2022 Aug 18
原文标识
PubMed 36010351 · DOI 10.3390/diagnostics12082001