皮肤原发性侵袭性亲表皮CD8阳性细胞毒性T细胞淋巴瘤一例  

Primary cutaneous aggressive epidermotropic CD8-positive cytotoxic T-cell lymphoma： a case report

作　　者：汪旸[1] 农琳[2] 武铃慎[1] 李挺[2] 涂平[1] 

机构地区：[1]北京大学第一医院皮肤性病科,100034 [2]北京大学第一医院病理科,100034

出　　处：《中华皮肤科杂志》2009年第4期237-240,共4页Chinese Journal of Dermatology

摘　　要：患者女，19岁。全身反复发作进行性溃疡伴发热1年。皮损初起为紫红色斑丘疹，迅速破溃形成溃疡，伴剧烈疼痛。皮损渐发展至躯干、四肢，伴间歇性高热。皮肤组织病理示真皮全层及皮下脂肪层结节状中至大异形淋巴样细胞浸润，伴局灶性亲表皮生长。浸润细胞免疫组化标记CD3、CD8、T细胞细胞内抗原、T细胞受体β均阳性。T细胞受体基因重排提示T细胞克隆性增生，确诊为皮肤原发性侵袭性亲表皮CD8阳性细胞毒T细胞淋巴瘤。患者通过环磷酰胺、长春新碱、泼尼松、博来霉素化疗，皮损部分好转，但仍于发病后22个月死亡。A 19-year-old Chinese woman presented with progressive ulcers and recurrent fever for 1 year. The lesions originally began with purplish red maculopapules, then rapidly developed into ulcer with severe pain and spread to the trunk and extremities accompanied by intermittent fever. Histopathology revealed lymphoid infiltration of middle and large atypical T cells throughout the dermis and subcutaneous fat layer along with focal epidermotropism. The lymphoid infiltrates were positive for CD3, CD8, T-cell intercellular antigen （TIA） and T-cell receptor β （TCR-β）. Gene analysis showed the rearrangement of T-cell receptor gene. According to the above findings, the patient was diagnosed as primary cutaneous aggressive epidermotropic CD8-positive cytotoxic T-cell lymphoma. Combined therapy with cyclophosphamide, vincristine, prednisone and Neomycin resulted in partial remission of skin lesions, but she eventually died 22 months after the onset of disease.

关 键 词：淋巴瘤 T细胞 皮肤 T淋巴细胞 细胞毒性 

分 类 号：R739.5[医药卫生—肿瘤]