Krokowski M, Winzer M, Lange K, Thorns C, Gafumbegete E, Feller A C, Merz H
Institut für Pathologie, Universitätsklinik zu Lübeck.
Hautarzt. 2003 Jun;54(6):536-40. doi: 10.1007/s00105-002-0443-8. Epub 2003 Jan 15.
Mycosis fungoides (MF) is a cutaneous T-cell lymphoma (CTCL) characterized by its typical progress in three stages: the patch-, the plaque- and the tumour-stage. The incidence of mycosis fungoides rises with age and the average age at presentation is about 50. Children and adolescents are rarely affected and there are only few reports in the literature. We report a 12- and a 15-year-old boy showing refractory skin lesions not typical for mycosis fungoides. The histo- and immunohistological investigations and the detection of clonal T-cell receptor gamma gene rearrangements confirmed the diagnosis of early onset mycosis fungoides in both cases.
蕈样肉芽肿(MF)是一种皮肤T细胞淋巴瘤(CTCL),其典型病程分为三个阶段:斑片期、斑块期和肿瘤期。蕈样肉芽肿的发病率随年龄增长而升高,发病时的平均年龄约为50岁。儿童和青少年很少受到影响,文献中仅有少数报道。我们报告了一名12岁和一名15岁男孩,他们表现出不典型的蕈样肉芽肿难治性皮肤病变。组织学和免疫组织学检查以及克隆性T细胞受体γ基因重排检测证实了这两例均为早发性蕈样肉芽肿。