皮肤 T 细胞淋巴瘤:单中心回顾性分析

Till Kaemmerer, Anne Guertler, Benjamin M Clanner-Engelshofen, Corbinian Fuchs, Lars Einar French, Markus Reinholz
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摘要

简介:皮肤 T 细胞淋巴瘤(CTCL皮肤T细胞淋巴瘤(CTCL)是一种罕见疾病,其特征是恶性T细胞浸润皮肤。我们评估了CTCL的发病率、流行病学和治疗方法,重点研究了其最著名的亚型,即真菌病(MF)和塞扎里综合征(SS):我们回顾性地分析了2015年1月至2019年12月这5年间在我院门诊部就诊并经组织学确诊为CTCL的患者的医疗数据:我们评估了102名患者的档案,其中67%为男性,33%为女性。总平均年龄为59.1±14.1(24-86)岁。92名患者(90%)被诊断为 MF,10名患者(10%)被诊断为 SS。根据ISCL/EORTC标准,大多数患者最初分为IA期(34%)和IB期(45%)。疾病发生率在晚期有所下降(II 期:4%;III 期:7%;IV 期:10%)。45 名患者(44.1%)只接受了皮肤导向疗法(SDT)。20名患者(19.6%)从SDT发展到全身治疗(ST)。37名患者(36.3%)从治疗一开始就接受了ST与SDT相结合的治疗(TS)。总的来说,有50种不同的TS治疗方法是由于缺乏治疗反应或疾病进展而启动的:CTCL的治疗旨在维持患者的生活质量,同时最大限度地减少副作用。由于 CTCL 通常是无法治愈的疾病,因此治疗的重点在于控制症状和预防疾病进展。由于患者群体大、观察时间长,我们的研究可以对德国一所大学门诊中 MF 和 SS 的发病频率和治疗方法进行有效评估。我们倾向于在早期阶段采用局部疗法,而在晚期阶段采用更具侵入性的疗法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Cutaneous T-cell Lymphomas: A Single-center Retrospective Analysis.

Introduction: Cutaneous T-cell lymphomas (CTCLs) are rare diseases characterized by infiltration of malignant T-cells into the skin. We evaluated the prevalence, epidemiology, and therapy of CTCLs, focusing on its most well-known subtypes, namely mycosis fungoides (MF) and Sézary syndrome (SS).

Patients and methods: We retrospectively analyzed the medical data of patients with a histologically confirmed diagnosis of CTCL presenting to our outpatient department during a 5-year period from January 2015 to December 2019.

Results: We evaluated the files of 102 patients, of whom 67% were men and 33% women. The overall mean age was 59.1±14.1 (24-86) years. Ninety-two patients (90%) were diagnosed with MF and ten patients (10%) with SS. According to ISCL/EORTC, the majority of patients initially classified as stage IA (34%) and IB (45%). Disease frequency decreased at advanced stages (II: 4%; III: 7%; IV: 10%). Forty-five patients (44.1%) received only skin-directed therapy (SDT). Twenty patients (19.6%) progressed from SDT to systemic therapy (ST). Thirty-seven patients (36.3%) received ST combined with SDT (TS) from the start of treatment. Overall, fifty different therapeutic approaches of TS were initiated due to lack of response to therapy or disease progression.

Conclusion: Management of CTCLs aims to maintain patient quality of life while minimizing side-effects. As CTCLs are usually incurable diseases, the focus of treatment is on symptom control and prevention of disease progression. Due to the large patient group and the long observation period, our study allows for a valid evaluation of the frequency and therapy of MF and SS in a university outpatient clinic in Germany. We favor topical therapies in early stages with more invasive therapies in advanced stages.

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