Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, Canada.
Department of Pathology, Sunnybrook Health Sciences Centre, Toronto, Canada.
Int J Dermatol. 2019 Aug;58(8):933-939. doi: 10.1111/ijd.14391. Epub 2019 Feb 14.
Mycosis fungoides (MF) typically has a CD4 CD8 T-cell phenotype. Rare cases of CD4 CD8 , CD4 CD8 , or CD4 CD8 immunophenotypes have been described. Little is known about the impact of MF immunophenotypes on disease behavior.
We conducted a retrospective cohort study to review all cases of MF from 2007 to 2017 from Sunnybrook Health Sciences Centre, Toronto, Canada. CD4 CD8 (Group 1) was compared to the three less common subtypes (Group 2) with respect to stage at diagnosis, progression, and transformation. Potential confounding factors (demographic, clinical, and laboratory parameters) were assessed.
A total of 160 patients with confirmed MF were analyzed, including 126 CD4 CD8 MF (79%), 26 CD4 CD8 MF (16%), six CD4 CD8 MF (4%), and two CD4 CD8 MF (1%). Both groups were similar with respect to demographics and laboratory parameters at the time of diagnosis. There was no difference between patients with late stage disease (10% vs. 9%) for groups 1 and 2, respectively (P = 0.901). There was no statistically significant difference either in 5-year progression (27.7% vs. 23.5%, P = 0.283) or transformation (16.2% vs. 17.3%, P = 0.350) estimates. We did find that atypical immunophenotypes presented with different clinical morphologies and were less likely to require systemic therapy.
Our large cohort study indicates that atypical MF immunophenotypes do not seem to influence prognosis. Hypopigmented MF was more frequent in the CD4 CD8 group while folliculotropic MF was exclusively seen in the CD4 CD8 group. We believe that cases of CD8 MF with aggressive behavior described in the literature represent misclassified primary cutaneous aggressive epidermotropic CD8 T-cell lymphoma. The small number of patients included in the study is a limiting factor.
蕈样肉芽肿（MF）通常具有 CD4 CD8 T 细胞表型。已经描述了罕见的 CD4 CD8 、 CD4 CD8 或 CD4 CD8 免疫表型病例。关于 MF 免疫表型对疾病行为的影响知之甚少。
我们进行了一项回顾性队列研究，以审查 2007 年至 2017 年期间来自加拿大多伦多桑尼布鲁克健康科学中心的所有 MF 病例。CD4 CD8 （第 1 组）与三种不太常见的亚型（第 2 组）在诊断时的阶段、进展和转化方面进行了比较。评估了潜在的混杂因素（人口统计学、临床和实验室参数）。
共分析了 160 例确诊 MF 患者，包括 126 例 CD4 CD8 MF（79%）、26 例 CD4 CD8 MF（16%）、6 例 CD4 CD8 MF（4%）和 2 例 CD4 CD8 MF（1%）。两组患者在诊断时的人口统计学和实验室参数相似。第 1 组和第 2 组晚期疾病患者的比例分别为 10%（n=13）和 9%（n=5），无统计学差异（P=0.901）。5 年进展（27.7% vs. 23.5%，P=0.283）和转化（16.2% vs. 17.3%，P=0.350）估计值也没有统计学差异。我们确实发现，非典型免疫表型表现出不同的临床形态，不太可能需要全身治疗。
我们的大型队列研究表明，非典型 MF 免疫表型似乎不会影响预后。CD4 CD8 组中色素减退型 MF 更为常见，而滤泡性 MF 仅见于 CD4 CD8 组。我们认为，文献中描述的具有侵袭性行为的 CD8 MF 病例代表了误诊的原发性皮肤侵袭性表皮 CD8 T 细胞淋巴瘤。研究中纳入的患者数量较少是一个限制因素。
