摘要目的 通过分析11例自身免疫病(AID)合并噬血细胞综合征(HPS)的临床特点,提高对该病的认识.方法 收集北京协和医院2004年1月-2009年6月住院的AID合并HPS者11例,回顾性分析其发生诱因、各系统受累表现、治疗及预后.结果 11例AID合并HPS患者中男性3例,女性8例,年龄12 74(30.7±18.3)岁.基础病:斯蒂尔病4例,系统性红斑狼疮(SLE)3例,干燥综合征(SS)、类风湿关节炎(RA)、韦格纳肉芽肿(WG)、克罗恩病(CD)各1例.诱发HPS因素:基础病活动4例,基础病活动并发感染6例,单纯感染1例.11例患者均有高热,其中肝脾肿大8例,淋巴结肿大7例,神经系统受累4例,并发弥漫性血管内凝血(DIC)4例.实验室检查:血细胞减少11例,肝功能异常11例,高甘油三酯血症5例,低纤维蛋白原血症9例,铁蛋白＞500 μg/L 6例,NK细胞活性降低4例.骨髓涂片均见吞噬血细胞现象.经大剂量糖皮质激素联合免疫抑制剂、积极抗感染、静脉人免疫球蛋白(IVIG)支持治疗,5例存活,6例死亡,合并DIC 4例均未存活(r=0.69,P=0.019).结论 AID并发HPS不易与活动性AID鉴别.合并DIC预后差、病死率高.糖皮质激素、免疫抑制剂及IVIG对HPS治疗有效,合并感染时加强抗感染治疗至关重要.

abstractsObjective To analyze the clinical features of patients with hemophagocytic syndrome (HPS) in autoimmune diseases (AID). Methods We collected the data of 11 patients with AID complicated with HPS in Peking Union Medical College Hospital from 2004 to 2009. The underlying diseases, clinical features, laboratory findings and treatment outcomes were retrospectively analyzed. Results Of the 11 patients,3 were male,8 were female. Mean age was (30. 7 ± 18. 3) years. The underlying diseases included Still disease ( n = 4 ), systemic lupus erythematosus ( n = 3 ), and rheumatoid arthritis, primary Sj(o)gren's syndrome, Wegener granulomatosis and Crohn disease in each one case. HPS was associated with the onset of AID ( n = 4), active infection alone ( n = 1 ) and both factors ( n = 6 ). HPS was clinically characterized by high fever ( 100% ), hepatosplenomegaly ( 72. 7% ) , lymphadenopathy ( 63.3% ) and central nervous system involvement (36. 3% ). 4 patients presented with disseminated intravascular coagulation(DIC) (36. 3% ). Laboratory data mainly manifested with cytopenia ( 100% ), liver dysfunction ( 100% ), hypofibrinogenemia ( 62. 5% ), hypertriglyceridemia ( 81.8% ), serum ferritin ＞ 500 μg/L (100%), low NK-cell activity(80% ) and hemophagocytosis in bone marrow( 100% ). Based on treating underlying infections and use of corticosteroids and immunosuppressive agents in combination with intravenous immunoglobulins(IVIG) therapy, 5 patients recovered , 6 patients died. The mortality rate was 54. 5%. DIC were associated with mortality ( r = 0. 69, P = 0. 019 ). Conclusion The episode of HPS always occurs simultaneously with multiple system involvement that was often difficult to distinguish from active AID. The present of DIC on HPS related with poor prognosis and high mortality. Corticosteroids and immunodepressant and IVIG may improve the prognosis of HPS, while anti-infection therapy is very important and necessary for the patients accompany with active infection.