Department of Hematology and Medical Oncology, Winship Cancer Institute of Emory University, Atlanta, Georgia.
Biostatistics Shared Resource, Winship Cancer Institute of Emory University, Atlanta, Georgia.
JAMA Dermatol. 2022 Nov 1;158(11):1293-1299. doi: 10.1001/jamadermatol.2022.3601.
Mycosis fungoides and Sézary syndrome (MF/SS) has an increased incidence in Black patients, but clinical characteristics, treatments, and outcomes have been poorly characterized.
To assess racial differences in presentation and outcome and identify drivers for racial disparities in MF/SS.
DESIGN, SETTING, AND PARTICIPANTS: A retrospective cohort analysis was conducted of 566 patients with MF/SS diagnosed from 1990 to 2020 and seen at the Winship Cancer Institute of Emory University and Grady Memorial Hospital, both in Atlanta, Georgia. Self-reported race and ethnicity were obtained from patient medical records and analyzed as 2 groups: non-Hispanic Black (Black) and all other races and ethnicities, including Asian, Hispanic, White, and unknown/undeclared (non-Black).
Univariate and multivariable models and Kaplan-Meier assessments were analyzed for overall survival and time to next treatment. The primary outcome was to assess differences in overall survival by racial and ethnic group. The hypotheses were formulated prior to data collection.
Of the 566 patients with MF/SS identified (mean [SD] age 55 [16.4] years; 270 (47.7%) female), 257 were Black and 309 were non-Black. Black race was associated with increased rates of progression to a higher TNMB stage (39.8% in Black patients vs 29.1% in non-Black patients; P < .001) but not survival. Black patients were younger and had increased female predominance, higher TNMB stage, higher tumor stage, nodal involvement, and higher lactate dehydrogenase level compared with non-Black patients with MF/SS. Hypopigmented MF (HMF) was found in 62 patients, who were mostly Black (n = 59). Hypopigmented MF was significantly associated with survival on univariate and multivariable models, with 10-year survival of 100% in patients with HMF compared with 51.8% in patients without HMF. Black race was only associated with inferior outcomes after excluding patients with HMF who were younger than 60 years (hazard ratio [HR], 1.61; 95% CI, 1.02-2.55; P = .04), but not in patients older than 60 years (HR, 1.20; 95% CI, 0.80-1.81; P = .37). On multivariate analysis, among the cohort without HMF who were younger than 60 years, Black race remained statistically significant when controlling for cancer stage and large-cell transformation (HR, 1.27; 95% CI, 1.08-2.87; P = .43).
In this cohort study, Black patients with MF/SS showed distinct clinical presentations and patterns of progression with heterogeneous outcomes depending on age at presentation and presence of HMF.
蕈样肉芽肿和塞扎里综合征（MF/SS）在黑种人群中的发病率增加，但临床特征、治疗方法和结果的描述都很差。
评估黑种人群在发病和转归方面的种族差异，并确定 MF/SS 中种族差异的驱动因素。
设计、地点和参与者：对 1990 年至 2020 年间在佐治亚州亚特兰大的埃默里大学温希普癌症研究所和格雷迪纪念医院诊断的 566 例 MF/SS 患者进行了回顾性队列分析。从患者的病历中获得了自我报告的种族和民族，并将其分为 2 组：非西班牙裔黑种人（黑人）和其他所有种族和民族，包括亚洲人、西班牙裔、白种人和未知/未申报（非黑人）。
分析了单变量和多变量模型以及 Kaplan-Meier 评估，以评估总生存率和下一次治疗时间。主要结局是评估按种族和民族分组的总生存率差异。假设是在数据收集之前提出的。
在确定的 566 例 MF/SS 患者中（平均[标准差]年龄 55[16.4]岁；270[47.7%]为女性），257 例为黑人，309 例为非黑人。黑人种族与更高的 TNMB 分期进展率增加相关（黑人患者为 39.8%，非黑人患者为 29.1%；P<0.001），但与生存率无关。与非黑人 MF/SS 患者相比，黑人患者更年轻，女性比例更高，TNMB 分期更高，肿瘤分期更高，淋巴结受累更多，乳酸脱氢酶水平更高。在 62 例患者中发现了色素减退性 MF（HMF），他们大多是黑人（n=59）。HMF 在单变量和多变量模型上均与生存显著相关，HMF 患者的 10 年生存率为 100%，而无 HMF 的患者为 51.8%。排除年龄小于 60 岁的 HMF 患者后，黑人种族仅与较差的预后相关（风险比[HR]，1.61；95%CI，1.02-2.55；P=0.04），但在年龄大于 60 岁的患者中则不然（HR，1.20；95%CI，0.80-1.81；P=0.37）。在多变量分析中，在年龄小于 60 岁且无 HMF 的队列中，黑人种族在控制癌症分期和大细胞转化后仍具有统计学意义（HR，1.27；95%CI，1.08-2.87；P=0.04）。
在这项队列研究中，患有 MF/SS 的黑人患者表现出明显的临床表现和进展模式，其结果因发病时的年龄和是否存在 HMF 而异，存在异质性。
