PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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).
UNASSIGNED: 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).
UNASSIGNED: Treatment with mogamulizumab was significantly and independently associated with decreased mortality in Sézary syndrome.
UNASSIGNED: French Society of Dermatology, Swiss National Science Foundation (IZLIZ3_200253/1) and SKINTEGRITY.CH collaborative research program.
UNASSIGNED: 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).
UNASSIGNED: 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).
UNASSIGNED: Treatment with mogamulizumab was significantly and independently associated with decreased mortality in Sézary syndrome.
UNASSIGNED: French Society of Dermatology, Swiss National Science Foundation (IZLIZ3_200253/1) and SKINTEGRITY.CH collaborative research program.
摘要：
■Sézary综合征是一种极为罕见且致命的皮肤T细胞淋巴瘤（CTCL）。Mogamulizumab,抗CCR4单克隆抗体，在一项针对CTCL的随机临床试验中，最近发现与无进展生存期增加相关。我们旨在评估Sézary综合征的OS和预后因素，包括莫加穆利珠单抗治疗，在现实生活中。
■来自2000年至2020年诊断为Sézary（ISCL/EORTCIV期）和Sézary前（IIIB期）综合征患者的数据来自欧洲的24个中心。年龄,疾病阶段,血浆乳酸脱氢酶水平，诊断时的血嗜酸性粒细胞增多,在多变量Cox比例风险比模型中分析接受的大细胞转化和治疗.本研究已在临床试验中注册（SURPASSe01研究：NCT05206045）。
■纳入了三百三十九名患者（58％的男性，诊断时的中位年龄为70岁，第一季度至第三季度，61-79）：塞萨里前33人（占339人的9.7%），296Sézary综合征（87.3％），其中10人（2.9%）发生了大细胞转化。110名患者接受了莫加穆利珠单抗治疗。中位随访时间为58个月(95%置信区间[CI]，53-68).5年OS为46.5%(95%CI,40.6%-53.3%)。多变量分析表明，年龄≥80岁与<50岁（HR：4.9，95％CI，2.1-11.2，p=0.001），和大细胞转化(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的因素。Mogamulizumab治疗与死亡率降低显著相关(HR:0.34,95%CI,0.15-0.80,p=0.013)。
■莫加穆利珠单抗治疗与Sézary综合征死亡率降低显著且独立相关。
■法国皮肤病学会，瑞士国家科学基金会(IZLIZ3_200253/1)和SKINTEGRITY。CH合作研究计划。
■来自2000年至2020年诊断为Sézary（ISCL/EORTCIV期）和Sézary前（IIIB期）综合征患者的数据来自欧洲的24个中心。年龄,疾病阶段,血浆乳酸脱氢酶水平，诊断时的血嗜酸性粒细胞增多,在多变量Cox比例风险比模型中分析接受的大细胞转化和治疗.本研究已在临床试验中注册（SURPASSe01研究：NCT05206045）。
■纳入了三百三十九名患者（58％的男性，诊断时的中位年龄为70岁，第一季度至第三季度，61-79）：塞萨里前33人（占339人的9.7%），296Sézary综合征（87.3％），其中10人（2.9%）发生了大细胞转化。110名患者接受了莫加穆利珠单抗治疗。中位随访时间为58个月(95%置信区间[CI]，53-68).5年OS为46.5%(95%CI,40.6%-53.3%)。多变量分析表明，年龄≥80岁与<50岁（HR：4.9，95％CI，2.1-11.2，p=0.001），和大细胞转化(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的因素。Mogamulizumab治疗与死亡率降低显著相关(HR:0.34,95%CI,0.15-0.80,p=0.013)。
■莫加穆利珠单抗治疗与Sézary综合征死亡率降低显著且独立相关。
■法国皮肤病学会，瑞士国家科学基金会(IZLIZ3_200253/1)和SKINTEGRITY。CH合作研究计划。
