Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas; Department of Dermatology, Wright State University Boonshoft School of Medicine, Dayton, Ohio.
Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas.
J Am Acad Dermatol. 2014 Dec;71(6):1117-26. doi: 10.1016/j.jaad.2014.07.049. Epub 2014 Sep 26.
Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, typically presents in middle-aged to elderly individuals.
We sought to study the demographics, clinicopathologic features, treatment response, and prognosis of patients with biopsy-proven MF diagnosed before 20 years of age.
Patients were identified from a prospectively collected database for retrospective analysis.
Of 1902 patients with MF, 34 had juvenile-onset MF: 41% were stage IA, 56% were stage IB, and 3% were stage IIB at diagnosis. The male to female ratio was 1.1:1. The median age of symptom onset was 9 years (range 3-19 years), with a delay in diagnosis between 1 month and 14 years. Patients primarily presented with hypopigmented (53%), hyperpigmented (29%), and pink-violaceous (41%) patches/plaques. Immunohistochemistry revealed 39% with CD8(+) immunophenotype, 67% of which had hypopigmented lesions. The phototherapy response rate in 21 patients was 81%. All patients who completely responded to narrowband ultraviolet B phototherapy had hypopigmented MF.
This is a single cancer center study.
Juvenile-onset MF presents with early-stage disease with an overrepresentation of hypopigmented MF and CD8(+) immunophenotype. Narrowband ultraviolet B is an effective treatment option for juveniles, especially for those with the hypopigmented variant.
蕈样肉芽肿（MF）是最常见的皮肤 T 细胞淋巴瘤，通常发生于中老年人。
我们旨在研究在 20 岁之前确诊的蕈样肉芽肿患者的人口统计学、临床病理特征、治疗反应和预后。
通过前瞻性收集的数据库，我们对患者进行了回顾性分析。
在 1902 例 MF 患者中，有 34 例为青少年发病型 MF：41%为 IA 期，56%为 IB 期，3%为 IIB 期。男女比例为 1.1:1。症状起始的中位年龄为 9 岁（范围 3-19 岁），诊断时存在 1 个月至 14 年的延迟。患者主要表现为色素减退（53%）、色素沉着（29%）和粉红色-紫色（41%）斑块/斑片。免疫组化显示 39%为 CD8+免疫表型，其中 67%为色素减退病变。21 例患者的光疗反应率为 81%。对窄谱紫外线 B 光疗完全有反应的患者均为色素减退型 MF。
这是一项单癌症中心研究。
青少年发病型 MF 表现为早期疾病，色素减退型 MF 和 CD8+免疫表型的比例过高。窄谱紫外线 B 是青少年的有效治疗选择，尤其是对色素减退型患者。
