种痘水疱病样淋巴瘤2例临床病理分析  

Hydroa vacciniforme-like cutaneous lymphoma: a clinicopathologic analysis of two cases

作　　者：唐雪峰[1] 郭乔楠[1] 

机构地区：[1]第三军医大学新桥医院病理科,重庆400037

出　　处：《临床与实验病理学杂志》2016年第8期878-881,共4页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨种痘水疱病样淋巴瘤(hydroa vacciniforme-like lymphoma,HVLL)的临床病理学特点、诊断及鉴别诊断。方法对2例HVLL进行临床资料分析、病理形态观察、免疫组化和EB病毒原位杂交检测、T细胞受体基因重排检测。结果 2例患者分别为15岁和18岁,均有10年以上的反复头部种痘水疱病样皮疹伴高热。肿瘤细胞浸润真皮或至皮下组织,小到中等大,轻中度异型性,围绕皮肤附件和嗜血管生长。2例肿瘤细胞CD2、CD3、CD7、CD8、TIA-1和Granzyme B均(+);CD56和CD30均(-)。2例EBER均(+)。例1标本TCR克隆性重排检测(+)。例1患者行CEOD方案化疗1次,确诊后1年因肺部感染、呼吸循环衰竭死亡。例2患者行化疗4次,病情得以控制。结论 HVLL是一种罕见的发生于儿童的EB病毒相关性T细胞淋巴瘤,临床病史尤其是皮损特点为诊断重要提示,结合病理形态、免疫表型、EB病毒原位杂交、T细胞受体基因重排可确诊。Purpose To analyze the clinical and pathological features of hydroa vacciniforme-like lymphoma（HVLL）. Methods The clinical data, pathological features, immunohistochemistry, detection of EBV by in situ hybridization, TCR gene rearrangement were analyzed in 2 cases of HVLL. Results The patients aged 15 years and 18 years, respectively. Both the patients had repeatedly hydroa vacciniforme-like skin lesions on the head over ten years, accompanying with fever. Histopathological examination showed a small or medium sized variable atypical lymphocytes infiltration in the dermis/subcutaneous, with periadnexal and perivascular involvements. Immunohistochemical staining showed the tumor cells were positive for CD2, CD3, CD7, CD8, TIA-1 and Granzyme B, and negative for CD56, and CD30. Both cases were EBER-positive and one case showed monoclonal T-cell receptor rearrangement. One patient died secondary to respiratory failure one year after diagnosis. Another patient remained alive after 4 courses of chemotherapy. Conclusion HVLL is a rare EBV-associated cutaneous T cell lymphoma that typically affects children. The special rash is important clue to diagnosis. The diagnosis is in combination with clinical manifestations, morphology, immunophenotype, detection of EBV, as well as TCR gene rearrangement analysis.

关 键 词：T细胞淋巴瘤 种痘水疱病 EB病毒 

分 类 号：R733.4[医药卫生—肿瘤]