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蕈样肉芽肿及其变异型:单中心93例中国患者的回顾性研究

Mycosis Fungoides and Variants of Mycosis Fungoides: A Retrospective Study of 93 Patients in a Chinese Population at a Single Center.

作者信息

Luo Yixin, Liu Zhaorui, Liu Jie, Liu Yuehua, Zhang Wei, Zhang Yan

机构信息

Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

出版信息

Ann Dermatol. 2020 Feb;32(1):14-20. doi: 10.5021/ad.2020.32.1.14. Epub 2019 Dec 27.

Abstract

BACKGROUND

Mycosis fungoides (MF) is the most common types of cutaneous T cell lymphoma. It typically presents with erythematous patches and plaques in the early stage and tumors and extracutaneous involvement with possibly fatal outcomes in the late stage. To facilitate early and accurate diagnosis of MF, it is essential to be knowledgeable of classic and variants of this disease. However, there is limited published data in the Chinese population.

OBJECTIVE

To characterize our patient group and to provide additional insight into these malignancies.

METHODS

Patients diagnosed with mycosis fungoides and its variants from October 2012 to January 2018 were retrospectively analyzed. Disease-specific survival (DSS) rate and curve according to early and advanced stages were also calculated.

RESULTS

The mean age at diagnosis of ninety-three MF patients was 38.9±1.73 years (range: 677). Forty-five males (48.4%) and 48 females (51.6 %) were included in this study. The DSS rate of early-stage MF was 98.6%, while that of advanced stage MF was 88.9%. There was a significant difference in DSS rate between early stage and advanced stage MF (=00.042, log-rank test). The median age of 10 patients with hypopigmented MF (hMF) was 10.5 years (range: 628). The age of hMF was younger than that of classical MF patients (<0.05).

CONCLUSION

Early-stage MF has a better prognosis than advanced stage and hMF affects younger people than classic MF among Chinese. This study provides an insight into mycosis fungoides and its variants in a Chinese population.

摘要

背景

蕈样肉芽肿(MF)是最常见的皮肤T细胞淋巴瘤类型。其早期通常表现为红斑和斑块,晚期则出现肿瘤及皮肤外受累,可能导致致命后果。为促进MF的早期准确诊断,了解该疾病的经典表现及变异型至关重要。然而,针对中国人群的已发表数据有限。

目的

描述我们的患者群体,并对这些恶性肿瘤提供更多见解。

方法

回顾性分析2012年10月至2018年1月诊断为蕈样肉芽肿及其变异型的患者。还计算了早期和晚期疾病特异性生存率(DSS)及曲线。

结果

93例MF患者的平均诊断年龄为38.9±1.73岁(范围:677岁)。本研究纳入45例男性(48.4%)和48例女性(51.6%)。早期MF的DSS率为98.6%,而晚期MF为88.9%。早期和晚期MF的DSS率存在显著差异(=00.042,对数秩检验)。10例色素减退性MF(hMF)患者的中位年龄为10.5岁(范围:628岁)。hMF患者的年龄低于经典MF患者(<0.05)。

结论

在中国,早期MF的预后优于晚期,且hMF累及的人群比经典MF患者年轻。本研究为中国人群中的蕈样肉芽肿及其变异型提供了见解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d82/7992633/5ebd0bb4af8b/ad-32-14-g001.jpg

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