CELL INTELLIGENCE · 肿瘤细胞治疗研究
肿瘤细胞治疗研究
原发性皮肤γδT 细胞淋巴瘤伴不寻常的临床病理特征：三例患者的分子特征研究
英文原题：Primary Cutaneous Gamma-Delta T-Cell Lymphoma With Unusual Clinicopathological Characteristics: A Study of Three Patients With Molecular Characterization.
查看英文原题
Primary Cutaneous Gamma-Delta T-Cell Lymphoma With Unusual Clinicopathological Characteristics: A Study of Three Patients With Molecular Characterization.
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中文摘要
原发性皮肤γδT细胞淋巴瘤（PCGDTCL）是一种罕见的细胞毒性淋巴瘤，具有相当大的临床病理异质性。我们报告了三例表现不寻常的患者，包括一例TCR沉默表型伴自发消退后快速进展、一例肉芽肿性变异型伴长期惰性病程，以及一例狼疮性脂膜炎样形式。分子研究证实了持续性克隆性疾病，并发现了涉及TET2、STAT3、CDKN2A、MAPK1、PDCD1和TNFAIP3的反复改变，且在疾病演变过程中获得了额外的突变。这些病例扩展了PCGDTCL的已知谱系，并强调了在诊断具有挑战性的表现中整合临床病理学和分子学发现的重要性。
展开英文摘要原文
Primary cutaneous gamma-delta T-cell lymphoma (PCGDTCL) is a rare cytotoxic lymphoma with considerable clinicopathological heterogeneity.
We report three patients with unusual presentations, including a TCR-silent phenotype with spontaneous regression followed by rapid progression, a granulomatous variant with a prolonged indolent course, and a lupus panniculitis-like form.
Molecular studies demonstrated persistent clonal disease and identified recurrent alterations involving TET2, STAT3, CDKN2A, MAPK1, PDCD1 and TNFAIP3, with acquisition of additional mutations during disease evolution. These cases expand the recognized spectrum of PCGDTCL and highlight the importance of integrating clinicopathological and molecular findings in diagnostically challenging presentations.
论文信息
- 作者
- Prieto-Torres L、Manso R、de la Pinta FJD、García MG、Moreno C、Martínez MAB、Briz AS、Rodríguez-Peralto JL
- 第一作者单位
- Dermatology Department, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.Spain
- 通讯作者单位
- Pathology Department, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.Spain
- 期刊
- The Australasian journal of dermatology2026 Aug 31
- 原文标识
- PubMed 42675786 · DOI 10.1111/ajd.70204