196名成人噬血细胞性淋巴组织细胞增多症患者的临床特征和预后分析  

Clinical manifestation and prognosis of 196 adult patients with hemophagocytic lymphohistiocytosis: analysis

作　　者：刘晨媛 涂盛[1] 赵瑞红 盛吉芳[1] LIU Chen-yuan;TU Sheng;ZHAO Rui-hong;SHENG Ji-fang(Department of Infectious Disease,The First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou,Zhejiang 310003,China;不详)

机构地区：[1]浙江大学医学院附属第一医院感染科,浙江杭州310003 [2]湖州市第一人民医院感染科,浙江湖州313000

出　　处：《中国微生态学杂志》2023年第2期202-206,共5页Chinese Journal of Microecology

摘　　要：目的 探究成人噬血细胞性淋巴组织细胞增多症(HLH)患者的临床特征及影响预后的独立危险因素。方法 收集2010—2021年浙江大学医学院附属第一医院感染科临床诊断为成人HLH的病例,应用HLH-2004诊断标准联合HScore评分对临床诊断病例重新进行诊断分析并入组。根据半年内随访结果,将患者分为存活组和死亡组两组,对入组患者病因、临床特点进行统计分析,明确影响成人HLH预后的影响因素。结果 纳入的196例确诊患者中,感染病例89例,恶性肿瘤82例,自身免疫性疾病18例,另外有7例病因不清。共157例患者完成随访,通过单因素分析发现,死亡组年龄更高,男性占比更高,黄疸及中枢神经系统症状发生率更高,且差异有统计学意义(P<0.05)。与存活组相比,死亡组入院CRP、肌酐、胱抑素、甘油三酯、胆红素、铁蛋白、Hscore更高(P<0.05),而血小板计数、血钠水平较低(P<0.05)。在治疗方面,生存组和死亡组是否选择含糖皮质激素、人免疫球蛋白或依托泊苷的治疗方案之间存在明显差异(P<0.01)。多因素回归分析发现,年龄增加、有中枢系统症状、肾小球滤过率降低和高Hscore评分是预后不良的独立危险因素(P<0.05)。结论 感染和恶性肿瘤是发生HLH的重要原因,而年龄增加、出现中枢系统症状、肾小球滤过率降低和高HScore评分可作为判断成人HLH预后不良的因素。Objective To explore the clinical manifestations and independent risk factors affecting the prognosis of adult patients with hemophagocytic lymphohistiocytosis(HLH). Methods Adult HLH cases clinically diagnosed in the department of infectious diseases of our hospital from 2010 to 2021 were collected and re-analyzed using HLH-2004 diagnostic criteria combined with HScore. According to the follow-up results within half a year, the patients were divided into survival group and death group. The etiology and clinical characteristics of the patients were statistically analyzed to determine the factors affecting the prognosis of adult HLH. Results A total of 196 patients were enrolled according to HLH-2004diagnostic criteria and HScore. In these cases, the etiology was varied, including 89 cases of infection, 82 cases of malignant tumor, 18 cases of autoimmune disease, and 7 cases of unknown etiology. Through univariate analysis, it was found that in death group the age was higher, the proportion of male was higher, and the incidence of jaundice and central nervous system symptoms was higher, with statistically significant difference(P<0.05). Compared with the survival group, the admission CRP, creatinine, cystatin, triglyceride, bilirubin, ferritin and Hscore in the death group significantly increased(P<0.05), while the platelet count and blood sodium significantly decreased(P<0.05). In terms of treatment, there was a significant difference between the survival group and the death group in whether to choose the treatment regimen containing glucocorticoid, human immunoglobulin or etoposide(P<0.01). Multivariate regression analysis showed that age, central system symptoms, decreased glomerular filtration rate and higher Hscore were independent risk factors for poor prognosis(P<0.05). Conclusion Infection and malignant tumor are the important causes of HLH, and increasing age, central system symptoms, decreased glomerular filtration rate and higher HScore can be used as the factors to determine poor prognosis of adult HLH

关 键 词：噬血细胞性淋巴组织细胞增多症 临床特征 HScore评分 预后 

分 类 号：R318.12[医药卫生—生物医学工程]