Novelli Silvana, García-Muret Pilar, Mozos Anna, Sierra Jorge, Briones Javier
a Hematology Service, Hospital de la Santa Creu i Sant Pau , Barcelona , Spain ;
b Dermatology Department , Hospital de la Santa Creu i Sant Pau , Barcelona , Spain ;
Leuk Lymphoma. 2016 May;57(5):1060-6. doi: 10.3109/10428194.2015.1057894. Epub 2016 Jan 11.
Mycosis fungoides and Sézary syndrome (MF/SS) are the most common forms of primary cutaneous T cell lymphomas. We analyzed the applicability of the cutaneous lymphoma international prognostic index (CLIPi) in MF/SS. We introduced the total body-surface area affected (TBSA) and the type of skin lesions at diagnosis as prognostic variables. The overall survival (OS) at median time of follow up (96 months) was 75.6% (CI 95%, 62.0-98.5%). In the univariate analysis, age>60 years, advanced disease, type of skin lesions and TBSA>50 showed poorer OS (p<0.05). In the multivariate analysis there was a significant increased relative risk of death in those patients>60 years, with advanced disease and TBSA>50% (p<0.05). TBSA identified a group of poor prognosis patients with advanced MF/SS that may benefit from novel systemic therapies.
蕈样肉芽肿和塞扎里综合征（MF/SS）是原发性皮肤T细胞淋巴瘤最常见的形式。我们分析了皮肤淋巴瘤国际预后指数（CLIPi）在MF/SS中的适用性。我们将诊断时受影响的全身表面积（TBSA）和皮肤病变类型作为预后变量。中位随访时间（96个月）的总生存率（OS）为75.6%（95%置信区间，62.0 - 98.5%）。单因素分析中，年龄>60岁、疾病晚期、皮肤病变类型以及TBSA>50%的患者总生存率较差（p<0.05）。多因素分析中，年龄>60岁、疾病晚期且TBSA>50%的患者死亡相对风险显著增加（p<0.05）。TBSA识别出一组预后较差的晚期MF/SS患者，这些患者可能从新型全身治疗中获益。
