Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy.
Laboratory of Immunogenetics, Department of Medical Sciences, University of Turin, 10126 Torino, Italy.
Cells. 2022 Jan 20;11(3):333. doi: 10.3390/cells11030333.
Sézary syndrome is a rare subtype of cutaneous T-cell lymphoma characterized by erythroderma, peripheral lymphadenopathies, and circulating atypical cerebriform T-cells. To date, no definite staging system has been developed for these patients. In this retrospective analysis of the archive of the Dermatological Clinic of the University of Turin, Italy, erythrodermic SS patients were classified according to clinical records and photographs into three main presentations: erythematous, infiltrated, or melanodermic. The pattern of erythroderma was found to be associated with disease outcome, as better survivals were recorded in patients with erythematous and infiltrative erythroderma. Patients in the melanodermic group, though less represented in our investigation, seemed to show a worse trend in survival. According to this preliminary evidence, a new prognostic classification, with a revised score specific for Sézary syndrome patients, can be proposed to usefully integrate the current staging system. The correlation displayed in our research will be hopefully confirmed by prospective studies with larger cohorts, with the aim of identifying significant prognostic features in this subset of cutaneous T-cell lymphoma patients.
蕈样肉芽肿病是一种罕见的皮肤 T 细胞淋巴瘤亚型,其特征为红皮病、外周淋巴结病和循环中出现非典型脑回状 T 细胞。迄今为止,尚未为这些患者制定明确的分期系统。在这项对意大利都灵大学皮肤科诊所档案的回顾性分析中,根据临床记录和照片,将红皮病蕈样肉芽肿病患者分为三种主要表现:红斑、浸润或色素沉着。红皮病的模式与疾病结局相关,红斑和浸润性红皮病患者的生存率较高。在我们的研究中,色素沉着组患者虽然较少,但生存趋势似乎较差。根据这初步证据,我们可以提出一种新的预后分类,即针对蕈样肉芽肿病患者的修订评分,以有效地整合当前的分期系统。我们的研究显示的相关性将有望通过更大队列的前瞻性研究得到证实,目的是在这组皮肤 T 细胞淋巴瘤患者中确定有意义的预后特征。