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目的:探讨儿童种痘样水疱病样EB病毒(Epstein-Barr virus,EBV)感染相关淋巴细胞增生性疾病的诊断与治疗。方法:回顾性分析我科收治的3例种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病患儿的临床资料、实验室检查、治疗及预后等临床资料,并对相关文献进行复习。结果:3例患儿幼年发病,均因面部、四肢等部位皮损反复发作而就诊,其中2例伴有发热、肝大及淋巴结肿大。皮损组织病理检查均提示真皮浅层、皮下组织及毛囊附属器周围有大量淋巴细胞浸润,部分有轻度异形,免疫组化示CD43、CD56、Ki-67阳性表达不一;3例患儿血液EBV抗体阳性,EBV-DNA复制活跃。通过糖皮质激素、抗病毒等药物等治疗后,有2例病情明显缓解,1例效果不佳。结论:种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病与EB病毒感染密切相关,具有特征性临床表现,通过糖皮质激素、抗病毒药物联合静脉丙种球蛋白治疗部分有效,但仍有发展为种痘样水疱病样皮肤T细胞淋巴瘤可能,需密切随访。
Objective: To investigate the diagnosis and treatment of lymphocytic proliferative diseases associated with Epstein-Barr virus (EBV) infection in children. Methods: The clinical data, laboratory examination, treatment and prognosis of 3 cases of IBD-like Epstein-Barr virus infection-associated lymphoproliferative diseases in our department were analyzed retrospectively. The related literatures were reviewed. Results: Three cases of infancy, were due to facial, limbs and other parts of skin lesions recurrent episodes and treatment, of which 2 cases accompanied by fever, hepatomegaly and lymph nodes. Histopathological examination showed that there were a large number of lymphocytic infiltration around the superficial dermis, subcutaneous tissue and follicular appendages, and some of them were mildly abnormal. Immunohistochemistry showed that the expression of CD43, CD56 and Ki-67 were different; EBV antibody positive blood, EBV-DNA replication is active. After treatment with glucocorticoids, antivirals and other drugs, 2 cases were significantly relieved and 1 case was ineffective. CONCLUSIONS: The disease associated with Epstein-Barr-like Epstein-Barr virus-like Epstein-Barr virus infection is closely related to Epstein-Barr virus (EBV) infection. It has the characteristic clinical manifestations that it is partly effective in the treatment of glucocorticoid and antiviral drugs combined with intravenous gamma globulin, As a kind of pock-like blister-like cutaneous T-cell lymphoma may require close follow-up.