Quaglino P, Zaccagna A, Verrone A, Dardano F, Bernengo M G
Section of Dermatology, Department of Medical and Surgical Specialties, University of Turin, Italy.
Dermatology. 1999;199(1):8-14. doi: 10.1159/000018196.
Mycosis fungoides (MF) is rare in young patients. Its clinical behavior is still uncertain, as some reports have suggested that it has a more aggressive course than does the adult-onset type.
To ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas.
Clinical, immunohistopathological and follow-up data of early-onset (<20 years of age) MF cases reported in the literature (n = 42) plus 7 described herein were compared with those of a cohort of adult-onset MF patients (n = 252) diagnosed at our institution since 1975.
The majority of the 49 early-onset MF patients had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The predominant phenotype was CD3+ CD4+CD7-CD8-. Seven patients had a stage progression, 6 with extracutaneous involvement. Five- and 10-year survival rates were 93 and 74%, respectively.
No statistically significant differences were found in the disease course between early- and adult-onset MF.
蕈样肉芽肿（MF）在年轻患者中较为罕见。其临床行为仍不明确，因为一些报告表明，与成人发病型相比，它的病程更具侵袭性。
确定早发型MF是否代表一组异质性皮肤T细胞淋巴瘤。
将文献报道的早发型（<20岁）MF病例（n = 42）加上本文描述的7例的临床、免疫组织病理学和随访数据，与自1975年以来在我们机构诊断的一组成人发病型MF患者（n = 252）的数据进行比较。
49例早发型MF患者中，大多数在诊断时处于斑块期疾病。10例有色素减退性皮损。主要表型为CD3 + CD4 + CD7 - CD8 - 。7例患者病情进展，6例有皮肤外受累。5年和10年生存率分别为93%和74%。
早发型和成人发病型MF在病程上未发现统计学上的显著差异。
