Abstract：
OBJECTIVE: Cutaneous T-cell lymphomas (CTCL) such as mycosis fungoides (MF) and Sézary syndrome (SS) are rare lymphomas with varying prognoses. The aim of the study was to describe the survival of a cohort of patients with MF/SS and evaluate the prognostic factors impacting disease survival.
METHODS: All cases of MF/SS diagnosed from 2008 through 2022 were retrospectively analyzed. The demographic variables, histological parameters, and analytical data were analyzed too. Progression-free survival (PFS) and disease-specific survival (DSS) were calculated.
RESULTS: A total of 148 cases were included. A total of 121 (82%) and 27 cases were diagnosed with MF, and SS, respectively. A total of 37 patients (25%) experienced progression at some point disease progression. The median PFS and median DSS were 127 and 135 months, respectively. Age >60 years, diagnosis of SS, the presence of large cell transformation (LCT) at diagnosis, folliculotropism in early stages, high Ki-67 expression, the presence of the clonal T-cell receptor (TCR) in blood, elevated LDH and B2M levels, and advanced stages (IIB, IVA, T3, T4, N3/Nx) were associated with worse prognosis across the entire cohort.
CONCLUSIONS: Stage IVA and the presence of LCT at diagnosis stood out as independent factors of unfavorable prognosis. LCT was the variable that most significantly impacted the patients\' survival and was closely associated with tumor skin involvement and stage IIB.
METHODS: All cases of MF/SS diagnosed from 2008 through 2022 were retrospectively analyzed. The demographic variables, histological parameters, and analytical data were analyzed too. Progression-free survival (PFS) and disease-specific survival (DSS) were calculated.
RESULTS: A total of 148 cases were included. A total of 121 (82%) and 27 cases were diagnosed with MF, and SS, respectively. A total of 37 patients (25%) experienced progression at some point disease progression. The median PFS and median DSS were 127 and 135 months, respectively. Age >60 years, diagnosis of SS, the presence of large cell transformation (LCT) at diagnosis, folliculotropism in early stages, high Ki-67 expression, the presence of the clonal T-cell receptor (TCR) in blood, elevated LDH and B2M levels, and advanced stages (IIB, IVA, T3, T4, N3/Nx) were associated with worse prognosis across the entire cohort.
CONCLUSIONS: Stage IVA and the presence of LCT at diagnosis stood out as independent factors of unfavorable prognosis. LCT was the variable that most significantly impacted the patients\' survival and was closely associated with tumor skin involvement and stage IIB.
摘要：
目的：皮肤T细胞淋巴瘤（CTCL）如真菌病（MF）和Sézary综合征（SS）是罕见的具有不同预后的淋巴瘤。该研究的目的是描述MF/SS患者队列的生存，并评估影响疾病生存的预后因素。
方法：对2008-2022年确诊的MF/SS病例进行回顾性分析。人口统计学变量，组织学参数，和分析数据也进行了分析。计算无进展生存期（PFS）和疾病特异性生存期（DSS）。
结果：共148例。共有121例(82%)和27例确诊为MF,和SS,分别。共有37名患者（25％）在某些疾病进展时经历了进展。中位PFS和中位DSS分别为127个月和135个月，分别。年龄>60岁，SS的诊断，诊断时存在大细胞转化（LCT），在早期阶段，Ki-67高表达,血液中存在克隆性T细胞受体（TCR），LDH和B2M水平升高，和高级阶段(IIB，IVA,T3，T4，N3/Nx）与整个队列中预后较差有关。
结论：IVA期和诊断时LCT的存在是预后不良的独立因素。LCT是最显著影响患者生存的变量，与肿瘤皮肤受累和IIB期密切相关。
方法：对2008-2022年确诊的MF/SS病例进行回顾性分析。人口统计学变量，组织学参数，和分析数据也进行了分析。计算无进展生存期（PFS）和疾病特异性生存期（DSS）。
结果：共148例。共有121例(82%)和27例确诊为MF,和SS,分别。共有37名患者（25％）在某些疾病进展时经历了进展。中位PFS和中位DSS分别为127个月和135个月，分别。年龄>60岁，SS的诊断，诊断时存在大细胞转化（LCT），在早期阶段，Ki-67高表达,血液中存在克隆性T细胞受体（TCR），LDH和B2M水平升高，和高级阶段(IIB，IVA,T3，T4，N3/Nx）与整个队列中预后较差有关。
结论：IVA期和诊断时LCT的存在是预后不良的独立因素。LCT是最显著影响患者生存的变量，与肿瘤皮肤受累和IIB期密切相关。
