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韩国单一三级中心 223 例蕈样肉芽肿患者的临床特征和长期预后:29 年回顾。

Clinical characteristics and long-term outcome of 223 patients with mycosis fungoides at a single tertiary center in Korea: A 29-year review.

机构信息

Department of Dermatology, Kosin University College of Medicine, Busan, Korea.

Department of Dermatology, Kosin University College of Medicine, Busan, Korea.

出版信息

J Am Acad Dermatol. 2022 Jun;86(6):1275-1284. doi: 10.1016/j.jaad.2021.06.860. Epub 2021 Jun 29.

DOI:10.1016/j.jaad.2021.06.860
PMID:34197872
Abstract

BACKGROUND

Data regarding Asian patients with mycosis fungoides (MF) are limited.

OBJECTIVE

We aimed to investigate the clinical profile and long-term outcomes of patients with MF in Korea.

METHODS

A retrospective review of 223 patients with MF who were followed up for more than 6 months or died of MF within 6 months of diagnosis was performed.

RESULTS

Approximately 96.4% and 3.6% of the patients had an early stage and advanced stage, respectively. The mean age at diagnosis was 44.8 years. The mean duration of symptoms before diagnosis was 47.0 months. Various subtypes were noted, including mycosis fungoides palmaris et plantaris (21.5%), folliculotropic (8.5%), pityriasis lichenoides-like (6.7%), ichthyosiform (4.0%), lichenoid purpura-like (2.7%), and hypopigmented (2.2%) MF. Juvenile patients accounted for 16.6%. The higher the skin T stage, the poorer the response to treatment. The 10-year overall survival was 96.8% in early-stage patients and 25.0% in advanced-stage patients. General prognosis was favorable, while recurrence and subtype switching were seen in 29.4% and 2.7% of patients, respectively.

LIMITATIONS

Our patients may not represent all Korean patients with MF.

CONCLUSION

MF in Korea has a high proportion of variants, a younger age at onset, and favorable prognosis. A high index of suspicion and skin biopsy are needed for early diagnosis.

摘要

背景

有关蕈样肉芽肿(MF)亚洲患者的数据有限。

目的

我们旨在研究韩国 MF 患者的临床特征和长期预后。

方法

对 223 例随访时间超过 6 个月或诊断后 6 个月内死于 MF 的 MF 患者进行回顾性分析。

结果

约 96.4%和 3.6%的患者分别处于早期和晚期。诊断时的平均年龄为 44.8 岁。诊断前症状的平均持续时间为 47.0 个月。注意到各种亚型,包括掌跖部蕈样肉芽肿(21.5%)、滤泡型(8.5%)、苔藓样糠疹样(6.7%)、鱼鳞样(4.0%)、苔藓样紫癜样(2.7%)和色素减退性 MF(2.2%)。青少年患者占 16.6%。皮肤 T 分期越高,治疗反应越差。早期患者的 10 年总生存率为 96.8%,晚期患者为 25.0%。总体预后良好,但 29.4%的患者出现复发,2.7%的患者出现亚型转换。

局限性

我们的患者可能无法代表所有韩国 MF 患者。

结论

韩国的 MF 具有较高的变异比例、发病年龄较小和良好的预后。需要高度怀疑并进行皮肤活检以实现早期诊断。

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Two Different Variants of Mycosis Fungoides (Hypopigmented Mycosis Fungoides and Mycosis Fungoides Palmaris et Plantaris) Occurring Simultaneously in a Single Patient.蕈样肉芽肿的两种不同变体(色素减退性蕈样肉芽肿和掌跖蕈样肉芽肿)同时出现在一名患者身上。
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