不同病因继发噬血细胞综合征的临床及实验室特征分析  

Clinical and laboratory characteristics of secondary hemophagocytic syndrome caused by different etiologies

作　　者：裴源源 姚然然 曹灵杰 杨烽涛 梁人戈 黄文凤[2] 朱继红[1] Pei Yuanyuan;Yao Ranran;Cao Lingjie;Yang Fengtao;Liang Renge;Huang Wenfeng;Zhu Jihong(Department of Rheumatology,Peking University People’s Hospital,Beijing 100044,China;Department of Emergency,Peking University People’s Hospital,Beijing 100044,China)

机构地区：[1]北京大学人民医院急诊科,北京100044 [2]北京大学人民医院风湿免疫科,北京100044

出　　处：《中华急诊医学杂志》2024年第7期999-1005,共7页Chinese Journal of Emergency Medicine

摘　　要：目的对继发性噬血细胞综合征(secondary hemophagocytic lymphohistiocytosis,sHLH)进行病因分类后,探讨其各自的临床、实验室及治疗特点,以深化疾病认识。方法连续收集北京大学人民医院2016年1月至2021年12月期间就诊的sHLH患者进行回顾性观察性研究,在排除年龄<18岁、临床资料缺失患者后,分析sHLH的就诊科室分布及病因分布,收集sHLH的基线资料、临床特点、并发症与实验室资料、治疗及院内结局,然后将sHLH患者按照病因分为肿瘤组、巨噬细胞活化综合征(macrophage activation syndrome,MAS)组及其他病因(以感染为主)组,组间比较采用卡方检验或方差分析、Mann-Whitney检验等检验方法。结果共纳入169例sHLH患者,其中27.8%为肿瘤相关sHLH,47.9%为MAS患者,24.3%为其他病因相关HLH以感染为主。对病因分类后进行统计学分析发现,其他原因组临床特点与肿瘤组更为一致,表现为外周血细胞减少更多及更重,sCD25表达更高,合并EB病毒感染更多,预后亦趋于一致,均有50%以上院内死亡;其他病因组中噬血现象最为多见(65.9%);而MAS组病死率较低,仅为17.3%(P<0.05),且治疗上激素冲击比例增高,环孢素A及白介素-2治疗比例显著升高。结论肿瘤相关HLH与其他病因(以感染为主)继发HLH临床特点及预后上表现更为相近,而MAS相对而言,整体病情较轻,预后较好。Objective To classify the etiology of secondary hemophagocytic syndrome(sHLH)and explore its clinical,laboratory and therapeutic characteristics in order to deepen the understanding of the disease.Method A retrospective observational study was conducted on sHLH patients who were treated at Peking University People's Hospital from January 2016 to December 2021.Patients under the age of 18 and those with missing clinical data were excluded.The distribution of departments visited and etiologies of sHLH were analyzed.Baseline data,clinical characteristics,complications,laboratory data,treatment,and in-hospital outcomes of sHLH were collected.The sHLH patients were then divided into 3 groups including malignancy group,macrophage activation syndrome(MAS)group and other etiologies(mainly infection)group.Intergroup comparisons were performed using chi-square tests,analysis of variance,Mann-Whitney tests,and other statistical methods.Results A total 169 patients were enrolled,among these patients,27.8%were malignancy-related HLH,47.9%were MAS,and 24.3%were other etiologies related HLH.Statistical analysis revealed that the clinical characteristics of other etiological group was highly consistent with the malignancy group,including more and severer peripheral blood cell reduction,higher sCD25 levels,more Epstein-Barr virus infection,and the prognosis was similar,both were with more than 50%in-hospital mortality.And the incidence of hemophagocytosis was highest in other etiological groups(65.9%).In contrast,MAS group was with an obviously lower mortality of 17.3%(P<0.05).Meanwhile,treatments including methylprednisolone pulse,cyclosporine A and interleukin-2 were used frequently in MAS group.Conclusion Malignancy related HLH and other etiologies related HLH exhibit more similar clinical characteristics and prognosis,while the MAS group,has a milder overall condition and better prognosis.

关 键 词：继发性噬血细胞综合征 巨噬细胞活化综合征 病因分布 感染 

分 类 号：R551.2[医药卫生—血液循环系统疾病]