坏死性淋巴结炎合并巨噬细胞活化综合征2例临床分析  

Macrophage activation syndrome preceded by necrotizing lymphadenitis in two children

作　　者：黄瑛[1] 何庭艳[1] 杨军[1] HUANG Ying;HE TingYan;YANG Jun(Shenzhen Children＇s Hospital,Shenzhen 518000,Guangdong,China)

机构地区：[1]深圳市儿童医院,广东深圳518000

出　　处：《临床儿科杂志》2018年第8期595-598,共4页Journal of Clinical Pediatrics

基　　金：深圳市科创委自由探索项目(No.JCY20170303155201082)

摘　　要：目的探讨坏死性淋巴结炎(KFD)合并巨噬细胞活化综合征(MAS)的临床特征、治疗及预后。方法回顾分析2例KFD合并MAS患儿的临床资料,并复习相关文献。结果 2例患儿均为男性,分别为14岁2个月和10岁7个月,均因发热入院。2例患儿颈部均可触及肿大淋巴结(最大分别为1.5 cm×0.5 cm和3 cm×3 cm),肝肋下1.5~2 cm。发热半个月左右出现乳酸脱氢酶、天门冬氨酸氨基转移酶及铁蛋白升高。淋巴结病理均显示淋巴结结构破坏,淋巴细胞坏死凋亡,CD 68和CD 123均为阳性。例1骨髓涂片可见较多组织细胞及噬血组织细胞,予地塞米松联合环孢素治疗后病情迅速缓解。例2骨髓涂片示少量组织细胞,偶见噬血组织细胞,予单纯口服泼尼松治疗后病情迅速缓解。结论 KFD出现持续高热2周以上,伴进行性铁蛋白和乳酸脱氢酶升高、纤维蛋白原逐渐下降,提示合并MAS可能,单用糖皮质激素或联合环孢素治疗有效,预后良好。Objective To explore the clinical phenotype, treatment and prognosis of macrophage activation syndrome（MAS） preceded by Kikuchi–Fujimoto disease（KFD）. Methods Clinical data of two children with MAS preceded by KFD were retrospectively analyzed, and related literatures were reviewed. Result Two cases, aged fourteen year two month and ten year seven month, respectively, were admitted due to fever. Palpation of the two boys showed enlargement of cervical lymph nodes and mild hepatosplenomegaly. Experimental examination revealed a progressively elevated levels of lactate dehydrogenase, aspartate aminotransferase and ferritin. Lymph node pathology showed destruction of lymph node structure and lymphocyte necrosis with positive histiocytic marker CD123 and CD68. Bone marrow smear of the first case showed a lot of tissue cells and hemophagocytic cells. Symptoms were soon quickly relieved with treatment of dexamethasone and cyclosporine. Bone marrow smear of the second case revealed a small amount of tissue cells and sparse hemophagocytic cells. Symptoms were soon quickly relieved with treatment of oral prednisone. Conclusions When KFD manifests as sustained high fever for more than two weeks, accompanied by progressively elevated levels of ferritin and lactate dehydrogenase, and gradually decreased level of fibrinogen, macrophage activation syndrome should be considered. Timely treatment using glucocorticoid or its combination with cyclosporine is effective and leads to good prognosis.

关 键 词：坏死性淋巴结炎 巨噬细胞活化综合征 细胞凋亡 

分 类 号：R725.5[医药卫生—儿科]