种痘水疱病样T细胞淋巴瘤的临床病理特征分析

Hydroa vacciniforme-like lymphoma:a clinicopathologic analysis with a review of literatures

目的观察种疸水疱病样T细胞淋巴瘤（HVLL）的临床病理特征，并探讨论该肿瘤的病理诊断。方法对5例HVLL病例进行了回顾性研究，包括临床病理表现、免疫组织化学染色、T细胞受体基因重排分析，以及采用原位杂交技术检测EB病毒感染情况等。结果病理学上，4个样本见表皮下水疱，3个样本有溃疡。所有病例均见中等大小的淋巴细胞在真皮层内胶原纤维之间，以及皮肤附件和血管周围浸润，3个有皮下组织浸润。免疫表型上，6个样本有CD4和CD8染色，其中CD8表型、CD4表型，以及CD4／CD8部分阳性各有2例。1／5个样本（16．13％）的瘤细胞表达cD56抗原。Ki-67阳性率为20％～60％。5个样本EBERI／2原位杂交检测均为阳性。5个样本均检出TCR克隆性重排。3例有EBV检查资料，均进行了EBV血清学和EBV载量检测，其中2例为EBV-EAD-IgG阳性，1例有EBV载量增加。5例皮肤种痘水疱病样T细胞淋巴瘤病例中2例有随访资料，其中一例死亡，生存时间分别为36和60个月。结论HVLL的临床病理表现有一定特征性，结合免疫表型及基因重排检测可明确诊断。该肿瘤需与一些皮肤良、恶性淋巴增生性疾病相鉴别。

Objective To observe the clinicopathologic feature of hydroa vacciniforme-like lymphoma （HVLL） and discuss the diagnosis of the tumor. Methods 5 patients of HVLL was retrospectively studied, with clinical and morphological review, immunohischemistry, T-cell receptor gene rearrangement analysis and in situ hybridization for Epstein- Barr virus （EBV） infection. Results The median age was 14 years （range 12-58）. 3 patients were children or adolescences, and the other two were adults. The patients included 2 males and 3 females. The medium course of disease was 60 months. 3 patients have vesicles and/or erythemas distributed on face, extremities and trunks. 2 patients had a fever, general condition was not bed. Three patients had the documents of EBV testing, Two of them were positive for EBV- EAD-IgG, and one had an increased EBV-DNA loading. Pathologically, subepidermal blisters and ulcerative lesion were observed in four and three samples, respectively. Small to medium-sized atypical lymphocyte infiltrated in epidermis, mainly perivascular and periadnexal distribution. In the 6 samples examined, CD8 phenotype, CD4 phenotype and CD4/ CD8 phenotype were 2, respectively. CD56 expression was found in one case. Ki-67 index was 20%-60%. EBER-ISH signals and TCR gene rearrangement were observed in all 7 samples. Follow-up data was available in 2 patients, one patient was dead, and the survival time was 36 and 60 months respectively. Conclusion There is a certain degree of specific in clinicopatological manifestation of HVLL, The diagnosis of the tumor is established based on the combination among clinocopathology, immunophenotype and genotype analysis, as well as the detection of EBV status. HVLL must be distinguished from a group of benign and malignant lymphoprolifcrative disorders of the skin.