获得性噬血细胞综合征临床特征及预后研究  

Study on clinical characteristics and prognosis of acquired hemophagocytic syndrome

作　　者：吴凡 张家奎 陶千山 朱维维 安福润 翟志敏 WU Fan;ZHANG Jiakui;TAO Qianshan;ZHU Weiwei;AN Furun;ZHAI Zhimin(Department of Hematology,the Second Hospital of Anhui Medical University/Hematologic Diseases Research Center,Anhui Medical University,Hefei,Anhui 230601,China)

机构地区：[1]安徽医科大学第二附属医院血液科/安徽医科大学血液病研究中心,合肥230601

出　　处：《重庆医学》2021年第4期591-594,共4页Chongqing medicine

基　　金：国家自然科学基金项目(81670179);安徽省高校自然科学研究重点项目(KJ2019A0274)。

摘　　要：目的探讨成人获得性噬血细胞综合征(HPS)的临床特征及早期死亡预后因素。方法回顾性分析45例成人获得性HPS患者的潜在诱因、临床表现、实验室检查,治疗选择和转归,并对早期死亡的危险因素进行分析。结果获得性HPS最常见诱因为肿瘤、感染,临床表现无特异性,91.11%的患者早期即出现肝功能异常,通过针对噬血及病因的治疗,28例患者(62.22%)好转,其中11例在中位随访7.8个月后仍存活。30 d死亡率为37.78%,危险因素分析表明高龄、弥散性血管内凝血(DIC)、肌酐(Cr)升高与30 d死亡有关。结论成人获得性HPS以肿瘤多见,对于临床上出现高热、脾大、铁蛋白高及早期肝损的患者需考虑该病可能,针对高龄、DIC、Cr升高的HPS患者应高度重视。Objective To investigate the clinical characteristics and the prognostic factors related to early death of adult acquired hemophagocytic syndrome(HPS).Methods A retrospective study was conducted on the potential causes,clinical manifestations,laboratory tests,treatment choices and outcomes of 45 adult patients with acquired HPS.And then the risk factors for early death were analyzed.Results Tumor and infection were the most common potential causes of acquired HPS,but the clinical characteristics were non-specific.A total of 41 cases were performed with liver dysfunction at an early stage.A total of 28 cases(62.22%)were changed for the better after the treatment with anti-HPS and removing of the underlying cause,and of which 11 cases were finally survived after a median follow-up of 7.8 months.The mortality within 30 days was 37.78%and were closely related to the advanced age,DIC and elevated creatinine.Conclusion Tumor and infection are the most common potential cause of adult acquired HPS.It is necessary to consider the possibility of acquired HPS for the patients with high fever,splenomegaly,high ferritin and early liver dysfunction.High attention are needed to paid for the acquired HPS cases with advanced age,DIC and elevated creatinine.

关 键 词：成年人 适应性免疫 淋巴组织细胞增多症 噬血细胞性 预后 

分 类 号：R449[医药卫生—诊断学]