儿童种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病的诊断与治疗3例

发布时间：2018-07-05 16:12

本文选题：EB病毒 + 种痘样水疱病　；参考：《临床皮肤科杂志》2017年01期

【摘要】：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。
[Abstract]:Objective: to investigate the diagnosis and treatment of Epstein-Barr virus (EBV) associated lymphoproliferative diseases in children. Methods: the clinical data, laboratory examination, treatment and prognosis of 3 children with EBV associated lymphoproliferative diseases were retrospectively analyzed, and the related literatures were reviewed. Results all of the 3 cases were diagnosed by repeated attacks of skin lesions in the face and extremities, 2 of which were accompanied by fever, hepatomegaly and lymphadenopathy. Histopathological examination showed that there were a large number of lymphocytes infiltrating in the superficial dermis, subcutaneous tissues and hair follicle appendages, and some of them had mild dysplasia. Immunohistochemical staining showed that the positive expression of CD43 and CD56 Ki-67 was not the same in 3 children with EBV antibody positive and EBV-DNA replication was active. After treatment with glucocorticoid, antiviral and other drugs, 2 cases had obvious remission and 1 case had poor effect. Conclusion: Epstein-Barr virus associated lymphoproliferative disease is associated with Epstein-Barr virus (EBV) infection and has characteristic clinical manifestations, which is partly effective by glucocorticoid, antiviral drugs and intravenous immunoglobulin. However, it is still possible to develop into vaccina-like vesicular cutaneous T cell lymphoma, which should be closely followed up.
【作者单位】：湖南省儿童医院皮肤科;
【分类号】：R752

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