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97 例蕈样肉芽肿韩国患者的临床特征和长期预后。

Clinical characteristics and long-term outcomes of 97 Korean patients with mycosis fungoides.

机构信息

Department of Dermatology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.

出版信息

Int J Dermatol. 2021 Dec;60(12):1510-1519. doi: 10.1111/ijd.15561. Epub 2021 May 15.

DOI:10.1111/ijd.15561
PMID:33991105
Abstract

BACKGROUND

Mycosis fungoides (MF) shows racial and regional differences in terms of clinical features. The availability of therapeutic options as well as drugs differs from one country to another. There are only limited data on the clinical characteristics and treatment experience of MF from South Korea.

METHODS

Medical records of 97 patients with MF were retrospectively analyzed to investigate clinical features, survivals, and prognostic factors. Assessment of prognostic variables was done using univariate Cox proportional hazard models.

RESULTS

Median age at time of diagnosis was 45 years. The median time from onset of skin lesion to diagnosis of MF was 36 months with a median follow-up period of 96 months. A number of clinical variants of MF were observed. Treatment mainly consisted of narrow-band UVB, systemic retinoids, methotrexate, chemotherapy, and regional radiotherapy. Complete remission was observed in 78% of patients with records on their clinical course. About 12% experienced disease progression. No clinical prognostic factor apart from TNM staging was identified.

CONCLUSION

Despite delay in diagnosis, most cases of MF in Korea were diagnosed in early stages. Prognosis of our patients was more favorable than those of other geographic regions as reported in previous studies. Good response to treatment, consisting mainly of phototherapy and radiation therapy, and relatively indolent clinical behavior of disease were observed in this homogeneous cohort of Korean patients with MF.

摘要

背景

蕈样肉芽肿(MF)在临床表现方面存在种族和地域差异。治疗方案和药物的可及性因国家而异。目前,关于韩国 MF 的临床特征和治疗经验的相关数据非常有限。

方法

回顾性分析了 97 例 MF 患者的病历,以调查其临床特征、生存情况和预后因素。使用单因素 Cox 比例风险模型评估预后变量。

结果

诊断时的中位年龄为 45 岁。从皮肤病变出现到 MF 诊断的中位时间为 36 个月,中位随访时间为 96 个月。观察到多种 MF 的临床变异型。治疗主要包括窄带 UVB、全身维 A 酸、甲氨蝶呤、化疗和局部放疗。有记录的患者中,78%达到完全缓解。约 12%的患者出现疾病进展。除 TNM 分期外,未发现其他临床预后因素。

结论

尽管存在诊断延迟,但韩国的大多数 MF 病例仍在早期诊断。与以往研究报道的其他地理区域相比,我们的患者预后更好。在这组同质的韩国 MF 患者中,观察到治疗反应良好,主要包括光疗和放疗,以及疾病相对惰性的临床行为。

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