Department of Dermatology, Emory University School of Medicine, Atlanta, Georgia.
Department of Biostatistics and Bioinformatics, Rollins School of Public Health, Emory University, Atlanta, Georgia.
J Am Acad Dermatol. 2015 Feb;72(2):276-85. doi: 10.1016/j.jaad.2014.10.019. Epub 2014 Nov 17.
Limited data exist on patients with mycosis fungoides (MF) and Sézary syndrome (SS) from the southeastern United States, a region with a high proportion of African Americans (AA).
We sought to determine clinical characteristics, prognostic factors, and survival of patients with MF/SS in a southeastern US cohort, compare with other cohorts, and validate proposed revisions in MF/SS staging.
This was a retrospective chart review of patients from an academic dermatology referral center (Atlanta, GA) from 1998 to 2013. Kaplan-Meier estimates were calculated for overall survival, disease-specific survival, and progression; univariate and multivariate Cox proportional hazard models were used for assessment of prognostic variables.
Of 393 patients, 55.2% were white, 43.3% AA, and 1.5% other; 52.7% were male and 47.3% female (ratio 1.1:1). Mean age was 53.6 years; mean age among AA was 48.9 years. In all, 19.6% died of disease; 21.9% experienced disease progression. Advanced TNMB classification, presence of a circulating clone without phenotypic evidence of blood involvement, and older age were predictors of poor disease-specific survival in the multivariate analysis, whereas AA race was not.
This study was from a single academic center.
Outcomes of our patients generally paralleled those of other geographic regions. MF/SS may affect younger patients and more women than previously recognized, particularly among AA. Survival among AA may be more favorable than that observed in prior reports. Our data support the validity of the staging criteria revisions for MF/SS.
来自美国东南部（一个非裔美国人比例较高的地区）的蕈样真菌病（MF）和塞扎里综合征（SS）患者的数据有限。
我们旨在确定美国东南部队列中 MF/SS 患者的临床特征、预后因素和生存情况，并与其他队列进行比较，以及验证 MF/SS 分期修订的有效性。
这是对来自亚特兰大（GA）学术皮肤科转诊中心的患者进行的回顾性图表审查，时间为 1998 年至 2013 年。使用 Kaplan-Meier 估计法计算总生存、疾病特异性生存和进展的情况；使用单变量和多变量 Cox 比例风险模型评估预后变量。
在 393 名患者中，55.2%为白人，43.3%为非裔美国人，1.5%为其他种族；52.7%为男性，47.3%为女性（比例为 1.1:1）。平均年龄为 53.6 岁；非裔美国人的平均年龄为 48.9 岁。共有 19.6%的患者死于疾病；21.9%的患者出现疾病进展。在多变量分析中，高级 TNMB 分类、存在无血液受累表型证据的循环克隆以及年龄较大是疾病特异性生存不良的预测因素，而非裔美国人种族则不是。
本研究来自单一学术中心。
我们患者的结果总体上与其他地理区域相似。MF/SS 可能会影响比以前认识到的更年轻的患者和更多的女性，尤其是非裔美国人。非裔美国人的生存率可能比之前报道的更有利。我们的数据支持 MF/SS 分期标准修订的有效性。
