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原发性皮肤γδ T 细胞淋巴瘤的临床异质性:一项 20 年单中心经验

英文原题:Clinical heterogeneity of primary cutaneous γδ T-cell lymphoma: a 20-year single-institution experience.

PubMed 2026/07/21(内容时间) Blood Res Q2 · IF 3.7(JCR 2025)

研究概要

PCGDTCL 似乎表现出比以往认识到的更广泛的临床和治疗异质性。部分患者的持久缓解提示,治疗反应性可能在整个疾病谱中有所不同。

研究思路结论见上方概要

原发性皮肤γδ T细胞淋巴瘤(PCGDTCL)是一种罕见的皮肤T细胞淋巴瘤,以γδ T细胞受体表达和细胞毒性表型为特征。历史上一直认为其高度侵袭性,中位生存期为15-30个月,且由于罕见而无标准化治疗。然而,新近报道描述了行为相对惰性的亚群。我们报告了20年单中心经验,以更好地界定PCGDTCL的临床谱系和治疗意义。

回顾性分析了2005年1月至2025年9月在三星医疗中心诊断为PCGDTCL的患者。收集了临床、病理和免疫表型特征,并经血液病理学复核确认。病理特征使用热图进行可视化,治疗过程使用游泳图进行可视化。肿瘤反应根据实体瘤疗效评价标准(RECIST)1.1版进行评估。

共识别出6例患者(中位年龄57岁)。所有患者均表现为皮肤病变,范围从局限性孤立性病变到多灶性皮肤受累。^18F-氟脱氧葡萄糖正电子发射断层扫描(FDG-PET)显示代谢活性异质性,最大标准化摄取值(SUVmax)范围从无摄取到8.9。免疫表型分析显示均一CD3阳性、T细胞受体γδ表达、βF1阴性,以及CD4、CD8和CD56表达不一。一线治疗包括观察、糖皮质激素、CHOP(环磷酰胺、多柔比星、长春新碱、泼尼松)和GDP(吉西他滨、地塞米松、顺铂)。CHOP和GDP实现了持久完全缓解。一例患者经历多次复发,后来出现类似蕈样肉芽肿的临床病理特征,生存13年。

展开英文摘要原文

PURPOSE: Primary cutaneous γδ T-cell lymphoma (PCGDTCL) is a rare cutaneous T-cell lymphoma defined by γδ T-cell receptor expression and a cytotoxic phenotype. It has historically been considered highly aggressive, with median survival of 15-30 months and no standardized treatment due to its rarity. However, emerging reports describe subsets with relatively indolent behavior. We report a 20-year single-institution experience to better define the clinical spectrum and therapeutic implications of PCGDTCL. METHODS: Patients diagnosed with PCGDTCL at Samsung Medical Center between January 2005 and September 2025 were retrospectively reviewed. Clinical, pathologic, and immunophenotypic features were collected and confirmed by hematopathology review. Pathologic features were visualized using heatmaps, and treatment courses were visualized using swimmer plots. Tumor response was evaluated according to Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1. RESULTS: Six patients were identified (median age 57 years). All presented with cutaneous lesions, ranging from localized solitary lesions to multifocal cutaneous involvement. ^18F-fluorodeoxyglucose positron emission tomography (FDG-PET) demonstrated heterogeneous metabolic activity, with maximum standardized uptake values (SUVmax) ranging from absent uptake to 8.9. Immunophenotyping showed uniform CD3 positivity, T-cell receptor γδ expression, βF1 negativity, and variable CD4, CD8, and CD56 expression. First-line therapies included observation, corticosteroids, CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), and GDP (gemcitabine, dexamethasone, cisplatin). Durable complete remissions occurred with CHOP and GDP. One patient experienced multiple relapses, later developed clinicopathologic features resembling mycosis fungoides, and survived for 13 years. CONCLUSION: PCGDTCL appears to show broader clinical and therapeutic heterogeneity than historically appreciated. Durable responses in selected patients suggest that treatment responsiveness may vary across the disease spectrum.

论文信息

作者
Han M、Yoon SE、Cho J、Kim SJ、Kim WS
第一作者单位
Division of Hematology-Oncology, Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea. milanhan200@gmail.com.South Korea
通讯作者单位
Division of Hematology-Oncology, Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea. wskimsmc@skku.edu.South Korea
期刊
Blood research2026 Jul 21
原文标识
PubMed 42477178 · DOI 10.1007/s44313-026-00147-0