Sézary syndrome (SS) is a rare primary cutaneous T-cell lymphoma variant. Despite various treatment options, it remains incurable, with a poor prognosis. There is an urgent need for additional descriptive research to enhance our understanding and treatment of SS. The aim of this retrospective register-based study was to outline patients’ demographic characteristics; investigate the clinical, histopathological, and molecular findings; and assess treatment effectiveness with a focus on time to next treatment (TTNT) and disease progression. Data on 17 patients with SS were obtained from the primary cutaneous lymphoma register in West Sweden between 2012 and 2024. The results revealed that not all patients exhibited the classical triad of symptoms at diagnosis, emphasizing the need for personalized diagnostic approaches. The median survival was only 2.1 years, which reflects the aggressive nature of SS. The longest median TTNT was observed in triple therapy involving retinoids, interferon alpha, and extracorporeal photopheresis (ECP). There was no significant difference in TTNT between various lines of treatment. Early initiation of ECP treatment did not result in improved outcomes. This study highlights the importance of combination therapy for improved outcomes and underscores the need for future studies to identify optimal treatment approaches.
Sézary综合征（SS）是一种罕见的原发性皮肤T细胞淋巴瘤亚型。尽管存在多种治疗方案，该病仍无法治愈且预后不良。亟需开展更多描述性研究以深化对SS的认知与治疗。本项基于登记数据的回顾性研究旨在：概述患者人口学特征；探究临床、组织病理学及分子生物学表现；评估治疗效果，重点关注至下次治疗时间（TTNT）与疾病进展。研究数据来源于2012年至2024年间西瑞典原发性皮肤淋巴瘤登记库中收录的17例SS患者。结果显示并非所有患者在确诊时均呈现典型三联征，这凸显了个体化诊断策略的必要性。中位生存期仅为2.1年，反映出SS的侵袭性本质。维甲酸、α干扰素联合体外光分离置换疗法（ECP）的三联方案显示出最长的中位TTNT。不同治疗线数间的TTNT无显著差异。早期启动ECP治疗并未改善临床结局。本研究证实联合治疗对改善预后的重要性，并强调未来需通过研究探索最优治疗方案。
肿瘤(癌症)患者之家