具有γ - δ (γδ)表型的惰性蕈样真菌病的临床谱:包括治疗和结果的单一机构回顾

N. A. Ufkes, R. Tao, A. S. Halwani, R. Miles, S. D. Cipriano, M. Bowling, S. Florell, D. Gaffney, D. A. Wada
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引用次数: 0

摘要

原发性皮肤γδ t细胞淋巴瘤(CGD-CTL)是一种罕见的恶性肿瘤,典型表现为侵袭性临床表型。目的回顾γδ型蕈样真菌病临床表现为惰性的病例。方法回顾性分析2018年至2022年在犹他大学治疗的γδ表型和无痛临床病程的MF患者。结果5例患者,年龄4 ~ 81岁。所有患者均表现为临床持续性斑块期或斑块期疾病,组织病理学通过免疫染色显示TCR δ阳性,主要为表皮性浸润。这些患者都不需要多药化疗的积极治疗。结论CGD-TCL患者中有一部分在临床病理上不同于典型CGD-CTL,而在临床上类似于无痛性MF。因此,我们更倾向于将这些罕见病例称为“γδ表型MF”。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Clinical spectrum of indolent mycosis fungoides with a gamma-delta (γδ) phenotype: a single institution review including therapy and outcomes

Clinical spectrum of indolent mycosis fungoides with a gamma-delta (γδ) phenotype: a single institution review including therapy and outcomes

Background

Primary cutaneous γδ T-cell lymphoma (CGD-CTL) is a rare malignancy that classically exhibits an aggressive clinical phenotype.

Objectives

Review cases of mycosis fungoides with a γδ phenotype that demonstrated an indolent clinical course.

Methods

Retrospective review of patients diagnosed with MF with a γδ phenotype and an indolent clinical course between 2018 and 2022 treated at the University of Utah.

Results

Five patients ages 4-81 years old were identified. All presented with clinically persistent patch or plaque-stage disease, and histopathology showed primarily epidermotropic infiltrates positive for TCR delta via immunostaining. None of these patients have required aggressive treatment with multiagent chemotherapy.

Conclusions

There exists a subset of indolent CGD-TCL patients who are clinicopathologically distinct from classic CGD-CTL and clinically resembling indolent MF. Therefore, we prefer the term “MF with γδ phenotype” for these rare cases.

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