Novelli Silvana, García-Muret Pilar, Sierra Jorge, Briones Javier
a Hematology Department and.
b Dermatology Department , Hospital de la Santa Creu i Sant Pau , Barcelona , Spain.
J Dermatolog Treat. 2016;27(2):179-81. doi: 10.3109/09546634.2015.1086479. Epub 2015 Sep 25.
Sézary syndrome (SS) is characterized by rapidly progressive disease and poor survival. Although there is no standard treatment for SS, allogeneic stem cell transplantation (alloSCT) is the only treatment available that may offer a long survival. Alemtuzumab, a humanized monoclonal antibody that targets CD52, has reported some efficacy in this disease.
To describe the experience with alemtuzumab treatment in patients with SS in our center.
A total of six patients received alemtuzumab subcutaneously at different dosing regimens.
The median time of follow-up after alemtuzumab was 6 months (range 3-29 months). The overall response rate was 83.3% (5/6) with 66.7% complete responses. The disease-free survival (DFS) at 6 months was 33.3%. Increased DFS was observed in patients undergoing an alloSCT after alemtuzumab treatment. The overall survival at 6 months was 60%.
Alemtuzumab is an effective treatment in advanced mycosis fungoides/SS for palliation of symptoms and may be useful as a bridge therapy before alloSCT in relapsed/refractory patients.
塞扎里综合征（SS）的特点是疾病进展迅速且生存率低。尽管SS尚无标准治疗方法，但异基因干细胞移植（alloSCT）是唯一可能带来长期生存的治疗手段。阿仑单抗是一种靶向CD52的人源化单克隆抗体，已有报道称其对此病有一定疗效。
描述我们中心使用阿仑单抗治疗SS患者的经验。
共有6例患者接受了不同给药方案的阿仑单抗皮下注射治疗。
阿仑单抗治疗后的中位随访时间为6个月（范围3 - 29个月）。总体缓解率为83.3%（5/6），完全缓解率为66.7%。6个月时的无病生存率（DFS）为33.3%。在阿仑单抗治疗后接受alloSCT的患者中观察到DFS有所提高。6个月时的总生存率为60%。
阿仑单抗是晚期蕈样肉芽肿/SS的一种有效治疗方法，可缓解症状，对于复发/难治性患者，可能作为alloSCT前的桥接治疗手段。
