儿童种痘水疱病样淋巴瘤二例报告附文献复习

Hydroa vacciniforme-like lymphoma： two cases report and literatures review

目的提高对儿童种痘水疱病样淋巴瘤（HVLL）的认识。方法结合文献资料，对2例HVLL患儿的临床表现、病理特征及治疗转归进行回顾性分析。结果2例患儿均为女孩，年龄分别为4岁和12岁，病程分别为1年和10年。临床表现为四肢及头面部多形性皮疹；病理组织细胞形态学检查示皮肤受损组织真皮及其皮下组织内有异形淋巴细胞浸润，免疫组织化学染色检查示浸润细胞为T／NK细胞来源，EB病毒编码的小RNA检查结果阳性。例1化疗后治疗效果差，病情反复；例2仅给予对症治疗，皮肤损伤逐渐减轻，2个月后皮疹完全消失。结论HVLL临床表现独特，确诊主要靠皮肤病理组织活检行细胞形态学和免疫组织化学染色检查，目前尚无特异性治疗方法，预后有待观察。

Objective Two children with hydroa vacciniforme-like lymphoma（HVLL） were reported for a better understanding of this disease. Methods The clinical manifestation, pathological characteristics, therapeutic outcomes of two children with HVLL were analyzed and presented by compared with what described in literatures. Results Two children were girls, who treated firstly in the hospital in May 2012, July 2012 and their duration were 1 years, more than 10 years respectively. Their clinical manifestations were both limbs and craniofacial polymorphous rashes. Pathological findings revealed that the dermis and subcuta- neous tissue were profiled by atypical lymphocytic infiltration. Immunohistochemistry showed that the infiltra- tion of cells from T/NK cell, and Epstein-Barr virus encoded small RNA （EBER） （ ＋ ）. Case 1 was treated with chemotherapy, but her condition continued to deteriorate. Case 2 just received symptomatic treatment, her skin lesions gradually reduced and rash disappeared completely 2 months later. Conclusion HVLL is found with special clinical manifestation, its diagnosis mainly depend on skin biopsy and immunohistochemistry, there is no specific treatment method now, and its prognosis still needs further research.