联用依托泊苷成功治疗儿童组织细胞吞噬性脂膜炎合并EB病毒感染一例并文献复习  

Use of etoposide in treatment of pediatric cytophagic histiocytic panniculitis coinfected with EpsteinBarr virus: a case report and literature review

作　　者：李莎[1] 欧榕琼[1] 檀卫平[1] 

机构地区：[1]中山大学孙逸仙纪念医院儿科,广州510120

出　　处：《新医学》2017年第1期31-36,共6页Journal of New Medicine

基　　金：广东省医学科研基金(B2013120)

摘　　要：目的探讨儿童组织细胞吞噬性脂膜炎(CHP)合并EB病毒感染的临床特征、诊断与治疗经验。方法报道1例婴儿期起病的CHP合并EB病毒感染患儿临床诊治经过,并以"儿童""CHP""EB病毒"为检索词,在PubMed、万方数据知识服务平台、中国期刊全文数据库、维普中文科技期刊数据库中检索,结合国内外报道的相关资料对该病进行文献复习。结果该例为2岁10月龄女童,反复皮下结节伴发热2年4个月,伴肝脾肿大、淋巴结肿大、全血细胞减少、肝功能异常、高甘油三酯血症、凝血功能障碍、血浆EB病毒DNA增多,骨髓细胞学检查见噬血细胞,皮肤活组织检查见"豆袋样细胞"。患儿在外院被拟诊为"结节性非化脓性脂膜炎并巨噬细胞活化综合征"并给予泼尼松、环孢素及甲氨蝶呤治疗,病情进展。收入中山大学孙逸仙纪念医院诊断为CHP合并EB病毒感染。确诊后予联用依托泊苷,患儿逐渐获得临床及病理学缓解,随访13个月未见复发。检索文献后收集到已被详细报道的CHP 13例,患儿年龄6个月~19岁、中位年龄11岁,其中既往有EB病毒感染4例,患儿多以发热、皮下结节起病,可伴有肝、脾、淋巴结肿大。治疗上多数予糖皮质激素(激素)及免疫抑制剂,国外3例中有2例联用依托泊苷治疗,病情缓解;国内尚无应用依托泊苷的报道。结论儿童CHP是一种少见的特殊类型脂膜炎,进展快,病死率高;合并EB病毒感染者更为罕见,误诊率及治疗难度增加,病死率上升,传统的激素及免疫抑制剂治疗效果欠佳,尽早联用依托泊苷有助于缓解病情。Objective To explore the clinicopathological characteristics,diagnosis and treatment of pediatric cytophagic histiocytic panniculitis （ CHP） complicated with Epstein-Barr （ EB） vims infection. Methods We reported clinical diagnosis and treatment of one child with CHP co-infected with EB vims. Lit-erature review was conducted through PubMed, Wanfang data, CNKI and Chongqing Vip using the keywords of child, CHP and EB vims. Results A 34-month-old girl presented with repeated subcutaneous nodules and fe-ver for 28 months, accompanied by hepatosplenomegaly, lymphadenopathy, pancytopenia, liver dysfunction, hypertriglyceridemia, coagulopathy and elevated serum level of EB virus DNA. Bone marrow aspiration detected the sign of hemophagocytosis. Skin biopsy revealed bean bag-like cells. The child was diagnosed with Weber- Christian panniculitis and macrophage activation syndrome in a local hospital. The disease progressed after treatment with prednisone, cyclosporine A and methotrexate. In Sun Yat-sen Memorial Hospital of Sun Yat-sen University, she was diagnosed with CHP complicated with EB vims infection. Subsequently, she was adminis-tered with etoposide, and clinical and pathological remission was gradually obtained. No recurrence was ob-served during 13 months of follow-up. Thirteen pediatric cases of CHP have been reported, aged 6 months to 19 years with a median age of 11 years. Four children had a history of EB vims infection. Upon onset of CHP, a majority of affected children developed fever and subcutaneous nodules as primary symptoms, accompanied by hepatosplenomegaly and lymphadenopathy. Adrenocortical hormone （ hormone） and immunosuppressors were commonly used for CHP treatment. Two among 3 cases obtained clinical remission after etoposide treatment, as reported in English literatures. Use of etoposide in treating CHP has not been reported in China. Conclusions Childhood CHP is a rare specific type of panniculitis. The incidence of CHP complicated with EB vims infec-tion is extremel

关 键 词：脂膜炎 组织细胞 儿童 依托泊苷 EB病毒 

分 类 号：R725.1[医药卫生—儿科]