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大细胞转化蕈样肉芽肿患者的疾病特征、预后和对治疗的反应:一项单中心回顾性研究。

Disease characteristics, prognosis, and response to therapy in patients with large-cell transformed mycosis fungoides: A single-center retrospective study.

机构信息

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennysylvania.

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennysylvania; Department of Hematology & Oncology, Thomas Jefferson University, Philadelphia, Pennysylvania.

出版信息

J Am Acad Dermatol. 2022 Jun;86(6):1285-1292. doi: 10.1016/j.jaad.2021.07.013. Epub 2021 Jul 14.

Abstract

BACKGROUND

Mycosis fungoides with large-cell transformation (MF-LCT) is associated with an aggressive clinical course, yet data comparing treatment outcomes in MF-LCT are sparse.

OBJECTIVE

To compare treatment outcomes and to determine disease prevalence and characteristics associated with survival in MF-LCT.

METHODS

A retrospective review was conducted of mycosis fungoides patients from 2012 to 2020 treated at Thomas Jefferson University. Patients with histopathologic diagnosis of MF-LCT were included. Treatment outcomes were assessed by mean changes in the modified Severity Weighted Assessment Tool (mSWAT) and stage.

RESULTS

Of 171 patients with mycosis fungoides, 23 (13.4%) had histologic diagnosis of MF-LCT. The overall 5-year survival rate for MF-LCT was 74% and was not significantly associated with sex, age, or initial stage at the time of MF-LCT diagnosis. Brentuximab vedotin showed the greatest mean decrease in mSWAT (-20.53) and stage progression (change in Δ stage: -0.4) in MF-LCT compared to oral bexarotene (ΔmSWAT: +4.51; Δstage: +0.27), skin-directed therapy (ΔmSWAT: -5.93; Δstage: -0.08), and chemotherapy (ΔmSWAT: +4.97; Δstage: +0.85).

LIMITATIONS

Single-center retrospective design, and patients often on multiple treatment modalities.

CONCLUSIONS

We report superior treatment outcomes for brentuximab vedotin compared to oral bexarotene, skin-directed therapy, and chemotherapy in MF-LCT in both early and advanced disease.

摘要

背景

蕈样肉芽肿伴大细胞转化(MF-LCT)与侵袭性临床病程相关,但比较 MF-LCT 治疗结果的数据很少。

目的

比较 MF-LCT 的治疗结果,并确定与生存相关的疾病流行率和特征。

方法

对 2012 年至 2020 年在托马斯杰斐逊大学接受治疗的蕈样肉芽肿患者进行回顾性研究。纳入有 MF-LCT 组织病理学诊断的患者。通过改良严重程度加权评估工具(mSWAT)和分期的平均变化来评估治疗结果。

结果

在 171 例蕈样肉芽肿患者中,有 23 例(13.4%)有 MF-LCT 的组织学诊断。MF-LCT 的总体 5 年生存率为 74%,与性别、年龄或 MF-LCT 诊断时的初始分期无关。与口服贝沙罗汀(mSWAT 变化:+4.51;分期变化:+0.27)、皮肤定向治疗(mSWAT 变化:-5.93;分期变化:-0.08)和化疗(mSWAT 变化:+4.97;分期变化:+0.85)相比, Brentuximab vedotin 在 MF-LCT 中显示出最大的 mSWAT 平均下降(-20.53)和分期进展(分期变化:-0.4)。

局限性

单中心回顾性设计,且患者经常接受多种治疗方式。

结论

我们报告了在 MF-LCT 中,与口服贝沙罗汀、皮肤定向治疗和化疗相比, Brentuximab vedotin 在早期和晚期疾病中具有更好的治疗结果。

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