CAJ | 학술논문

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.
목적 제고대인동충두수포병양림파류(HVLL)적인식.방법 결합문헌자료,대2례HVLL환인적림상표현、병리특정급치료전귀진행회고성분석.결과 2례환인균위녀해,년령분별위4세화12세,병정분별위1년화10년.림상표현위사지급두면부다형성피진；병리조직세포형태학검사시피부수손조직진피급기피하조직내유이형림파세포침윤,면역조직화학염색검사시침윤세포위T/NK세포래원,EB병독편마적소RNA검사결과양성.례1화료후치료효과차,병정반복；례2부급여대증치료,피부손상축점감경,2개월후피진완전소실.결론 HVLL림상표현독특,학진주요고피부병리조직활검행세포형태학화면역조직화학염색검사,목전상무특이성치료방법,예후유대관찰.
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 subcutaneous tissue were profiled by atypical lymphocytic infiltration.Immunohistochemistry showed that the infiltration of cells from T/NK cell,and Epstein-Banr 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.