免疫疗法治疗蕈样肉芽肿综合征的实际疗效：一项多中心观察性队列研究。
Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.
作者信息
Bozonnat Alizée, Beylot-Barry Marie, Dereure Olivier, D'Incan Michel, Quereux Gaëlle, Guenova Emmanuella, Perier-Muzet Marie, Dalle Stephane, Grange Florent, Viguier Manuelle-Anne, Ram-Wolff Caroline, Feldmeyer Laurence, Beltraminelli Helmut, Bonnet Nathalie, Amatore Florent, Maubec Eve, Franck Nathalie, Machet Laurent, Chasset François, Brunet-Possenti Florence, Bouaziz Jean-David, Battistella Maxime, Donzel Marie, Pham-Ledard Anne, Bejar Claudia, Moins-Teisserenc Hélène, Mourah Samia, Saiag Philippe, Hainaut Ewa, Michel Catherine, Bens Guido, Adamski Henri, Aubin François, Boulinguez Serge, Joly Pascal, Tedbirt Billal, Templier Isabelle, Troin Laura, Montaudié Henri, Ingen-Housz-Oro Saskia, Faiz Sarah, Mortier Laurent, Dobos Gabor, Bagot Martine, Resche-Rigon Matthieu, Montlahuc Claire, Serret-Larmande Arnaud, de Masson Adèle
机构信息
Department of Dermatology, Saint-Louis Hospital, AP-HP, Paris, France.
INSERM U976, Institut de Recherche Saint-Louis, Paris, France.
出版信息
EClinicalMedicine. 2024 Jun 21;73:102679. doi: 10.1016/j.eclinm.2024.102679. eCollection 2024 Jul.
BACKGROUND
Sézary syndrome is an extremely rare and fatal cutaneous T-cell lymphoma (CTCL). Mogamulizumab, an anti-CCR4 monoclonal antibody, has recently been associated with increased progression-free survival in a randomized clinical trial in CTCL. We aimed to evaluate OS and prognostic factors in Sézary syndrome, including treatment with mogamulizumab, in a real-life setting.
METHODS
Data from patients with Sézary (ISCL/EORTC stage IV) and pre-Sézary (stage IIIB) syndrome diagnosed from 2000 to 2020 were obtained from 24 centers in Europe. Age, disease stage, plasma lactate dehydrogenases levels, blood eosinophilia at diagnosis, large-cell transformation and treatment received were analyzed in a multivariable Cox proportional hazard ratio model. This study has been registered in ClinicalTrials (SURPASSe01 study: NCT05206045).
FINDINGS
Three hundred and thirty-nine patients were included (58% men, median age at diagnosis of 70 years, Q1-Q3, 61-79): 33 pre-Sézary (9.7% of 339), 296 Sézary syndrome (87.3%), of whom 10 (2.9%) had large-cell transformation. One hundred and ten patients received mogamulizumab. Median follow-up was 58 months (95% confidence interval [CI], 53-68). OS was 46.5% (95% CI, 40.6%-53.3%) at 5 years. Multivariable analysis showed that age ≥ 80 versus <50 (HR: 4.9, 95% CI, 2.1-11.2, p = 0.001), and large-cell transformation (HR: 2.8, 95% CI, 1.6-5.1, p = 0.001) were independent and significant factors associated with reduced OS. Mogamulizumab treatment was significantly associated with decreased mortality (HR: 0.34, 95% CI, 0.15-0.80, p = 0.013).
INTERPRETATION
Treatment with mogamulizumab was significantly and independently associated with decreased mortality in Sézary syndrome.
FUNDING
French Society of Dermatology, Swiss National Science Foundation (IZLIZ3_200253/1) and SKINTEGRITY.CH collaborative research program.
背景
Sezary综合征是一种极其罕见且致命的皮肤T细胞淋巴瘤（CTCL）。莫加莫单抗是一种抗CCR4单克隆抗体，最近在一项CTCL随机临床试验中显示与无进展生存期延长相关。我们旨在评估在现实环境中Sezary综合征的总生存期（OS）和预后因素，包括莫加莫单抗治疗情况。
方法
收集2000年至2020年期间在欧洲24个中心诊断为Sezary（ISCL/EORTC IV期）和Sezary前期（IIIB期）综合征患者的数据。在多变量Cox比例风险模型中分析年龄、疾病分期、血浆乳酸脱氢酶水平、诊断时的血液嗜酸性粒细胞增多、大细胞转化和接受的治疗。本研究已在ClinicalTrials注册（SURPASSe01研究：NCT05206045）。
结果
共纳入339例患者（58%为男性，诊断时中位年龄70岁，四分位数间距为61 - 79岁）：33例为Sezary前期（占339例的9.7%），296例为Sezary综合征（占87.3%），其中10例（2.9%）发生大细胞转化。110例患者接受了莫加莫单抗治疗。中位随访时间为58个月（95%置信区间[CI]，53 - 68）。5年时OS为46.5%（95% CI，40.6% - 53.3%）。多变量分析显示，年龄≥80岁与<50岁相比（风险比[HR]：4.9，95% CI，2.1 - 11.2，p = 0.001），以及大细胞转化（HR：2.8，95% CI，1.6 - 5.1，p = 0.001）是与OS降低相关的独立且显著因素。莫加莫单抗治疗与死亡率降低显著相关（HR：0.34，95% CI，0.15 - 0.80，p = 0.013）。
解读
莫加莫单抗治疗与Sezary综合征死亡率降低显著且独立相关。
资助
法国皮肤病学会、瑞士国家科学基金会（IZLIZ3_200253/1）和SKINTEGRITY.CH合作研究项目。
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