自身免疫病相关性噬血细胞性淋巴组织细胞增多症16例临床特征  

Clinical features of 16 cases of autoimmune associated hemophagocytic lymphohistiocytosis

作　　者：任飞凤 陈林[2] 黄文瀚[1] 罗蕾 周俊 黄冬梅 沈敏 唐琳[1] REN Fei-feng;CHEN Lin;HUANG Wen-han;LUO Lei;ZHOU Jun;HUANG Dong-mei;SHEN Min;TANG Lin(Department of Rheumatology,The Second Affiliated Hospital,Chongqing Medical University,Chongqing 400010,China;Department of Hematopathology,The Second Affiliated Hospital,Chongqing Medical University,Chongqing 400010,China;Department of Rheumatology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Key Laboratory of Rheumatology and Clinical Immunology,Ministry of Education,Beijing 100730,China)

机构地区：[1]重庆医科大学附属第二医院风湿免疫科,重庆400010 [2]重庆医科大学附属第二医院血液科,重庆400010 [3]中国医学科学院,北京协和医学院,北京协和医院风湿免疫科风湿免疫病学教育部重点实验室,北京100730

出　　处：《中华临床免疫和变态反应杂志》2019年第1期11-16,共6页Chinese Journal of Allergy & Clinical Immunology

基　　金：国家自然科学基金面上项目(81771738)。

摘　　要：目的探讨自身免疫病相关性噬血细胞性淋巴组织细胞增多症(autoimmune associated hemophagocytic lymphohistiocytosis,AAHLH)患者的临床特点。方法收集并整理16例AAHLH患者的基础疾病、并发感染情况、临床、实验室检查、治疗及预后情况。结果基础疾病:系统性红斑狼疮(systemic lupus erythematosus,SLE)7例,类风湿关节炎(rheumatoid arthritis,RA)1例,SLE并发RA 1例,原发性干燥综合征(primary Sjogren's syndrome,pSS)1例,皮肌炎(dermatomyositis,DM)1例,未分化结缔组织病(undifferentiated connective tissue disease,UCTD)5例。部分患者同时并发感染,包括病毒感染4例,细菌感染3例,混合感染7例,感染部位以肺部为主。临床表现:高热(100%)、肝肿大(18.8%)、脾肿大(68.8%)、淋巴结肿大(75%)、皮疹(62.5%)、关节炎(68.8%),并发弥漫性血管内凝血(disseminated intravascular coagulation,DIC)3例(18.8%)。实验室检查:血细胞减少(100%)、肝功能异常(87.5%)、高甘油三脂血症(100%)、低纤维蛋白原血症(43.8%)、铁蛋白>500μg/L(100%)、可溶性CD25(sCD25)≥2400 U/ml(75%)、NK细胞活性降低(100%)。骨髓检查有成熟的单核巨噬细胞吞噬血细胞现象占56.3%。经过积极抗感染、治疗原发病以及经人免疫球蛋白、糖皮质激素、免疫抑制剂治疗,存活12例,病情恶化自动出院3例后死亡,在院死亡1例。结论AAHLH临床表现多样,容易并发感染,病死率高。sCD25水平高可能是AAHLH预后的不良因素。Objective To analyze the clinical features of patients with autoimmune associated hemophagocytic lymphohistiocytosis(AAHLH).Methods To collect and collate the basic diseases,co-infection,clinical,laboratory examination,treatment,and prognosis of diseases of 16 patients with AAHLH.Results The basic diseases include systemic lupus erythematosus(SLE)(n=7),rheumatoid arthritis(RA)(n=1),SLE complicated with RA(n=1),primary Sjogren's syndrome(n=1),dermatomyositis(n=1),undifferentiated connective tissue disease(n=5).Some patients combined with infection at the same time,including:4 cases of viral infection,3 cases of bacterial infection,7 cases of mixed infection,the infection site was mainly in the lungs.Clinical manifestations:high fever(100%),hepatomegaly(18.8%),splenomegaly(68.8%),lymphadenopathy(75%),skin rashes(62.5%),and arthritis(68.8%)and also 3 patients with disseminated intravascular coagulation(DIC)(18.8%).Laboratory tests showed:hematopenia(100%),hepatic insufficiency(87.5%),hypertriglyceridemia(100%),hypofibrinogenemia(43.8%),serumferritin>500μg/L(100%),soluble CD25(sCD25)≥2400 U/ml(75%),and NK cell activity decreased(100%).Bone marrow examination showed the phagocytosis of mature mononuclear macrophages,accounting for 56.3%.After active anti-infection,treatment of primary disease and treatment with human immunoglobulin,glucocorticoid and immunosuppressive agents,12 patients survived,3 patients died after their condition deteriorated and 1 patient died in hospital.Conclusions AAHLH has a variety of clinical manifestations,is prone to co-infection,and has a high fatality rate.High sCD25 level may be a prognostic factor for AAHLH.

关 键 词：自身免疫病相关性噬血细胞性淋巴组织细胞增多症 临床特点 治疗 

分 类 号：R59[医药卫生—内科学]