伴大细胞转化的蕈样肉芽肿1例

A Case of Large Cell Transformation of Mycosis Fungoides

患者女,61岁,左上肢红斑、丘疹16年,泛发全身7年,面部糜烂1月余。皮肤科情况:右侧顶部头皮、颜面部可见散在蚕豆至鸽蛋大小的红色斑块伴糜烂、渗出。全身可见广泛色素沉着、散在苔藓样斑块。皮损组织病理检查示:表皮棘层轻度肥厚,部分真皮乳头胶原纤维均质化,真皮浅层较多淋巴细胞浸润,其中部分淋巴细胞较大,有异型性,大细胞所占比例大于25%。免疫组化标记:瘤细胞CD3,CD3ε,CD4均(+),CD30部分(+),CD8,CD56,TIA-1,GrB均(-),Ki-67约50%阳性。EBER1/2原位杂交阴性。TCR-γ基因重排检测有克隆性重排条带。符合蕈样肉芽肿大细胞转化。

A 61-year-old man had suffered by erythema and papules since the age of 45 years, at first localized on the left upper limb and then slowly spreading to most of the body 7 years, with facial erosion for more than one month. Physical examination revealed a number of fava bean to pigeon egg sized red plaques with erosions scattered on the face and the top right scalp. Extensive pigmentation and hchenoid plaques were present over the whole body. Histopathological examination revealed mild acanthosis, papillary dermis collagen fibers of homogenization, and lymphocyte infiltration in the dermal superficial layer. More than 25% of lymphocytes were enlarged and typical. Immunohistochemistrical analysis revealed that all lymphocytes were CD3 ＋ , CD3e ＋ ,CD4 ＋ ,but CD8-,CD56-,TIA-l-,and GrB-,some of which were CD30 ＋ and Ki-67 ＋. EBER 1/2 in situ hybridization was negative. Clonal rearrangement band was detected for TCR-γ gene. All of this supported a diagnosis of mycosis fungoides with large cell transformation.