噬血细胞综合征早期死亡成年患者临床特征分析  

Analysis of clinical characteristics of early death in adult patients with hemophagocytic syndrome

作　　者：葛仁英[1] 刘盼 熊婷 张欣[2] 隗佳[3] Ge Renying;Liu Pan;Xiong Ting;Zhang Xin;Wei Jia(Department of Hematology,Xianning Central Hospital,Affiliated Hospital of Hubei University of Science and Technology,Xianning 437100,China;Department of Hematology,Huangshi Central Hospital,Huangshi 435000,China;Department of Hematology,Tongji Hospital Affiliated to Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,China)

机构地区：[1]咸宁市中心医院湖北科技学院附属第一医院血液内科,咸宁437100 [2]黄石市中心医院血液内科,黄石435000 [3]华中科技大学同济医学院附属同济医院血液内科,武汉430030

出　　处：《白血病．淋巴瘤》2023年第4期226-229,共4页Journal of Leukemia & Lymphoma

摘　　要：目的探讨噬血细胞综合征(HPS)早期死亡成年患者的临床特点。方法回顾性分析2016年9月至2021年11月咸宁市中心医院、黄石市中心医院及华中科技大学同济医学院附属同济医院53例成年HPS患者的临床资料。将患者根据确诊后28 d内是否死亡分组,比较两组患者的临床特征。采用logistic回归对患者28 d内死亡进行多因素分析。结果53例成年HPS患者28 d内死亡率为28.3%(15/53)。患者生存时间与白细胞计数(r=0.324,P=0.018)、总胆红素水平(r=-0.280,P=0.042)、清蛋白水平(r=0.281,P=0.042)均有关,但不存在线性因果关系(均P>0.05)。28 d内死亡组与非28 d内死亡组间患者年龄、血小板计数、清蛋白水平、肌酸激酶同工酶水平、三酰甘油水平、铁蛋白水平、是否累及中枢神经系统比较,差异均有统计学意义(均P<0.05)。多因素logistic回归分析显示,血小板计数<30×10^(9)/L、清蛋白<30 g/L、累及中枢神经系统、铁蛋白≥10000 ng/ml均为患者28 d内死亡的独立危险因素(均P<0.05)。结论对于成年HPS患者,根据铁蛋白水平、血小板计数、清蛋白水平、神经系统症状等评估早期死亡风险,积极纠正内环境紊乱,加强脏器支持治疗,有助于生存获益。Objective To explore the clinical characteristics of early death in adult patients with hemophagocytic syndrome(HPS).Methods The clinical data of 53 adult HPS patients in Xianning Central Hospital,Huangshi Central Hospital and Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology from September 2016 to November 2021 were retrospectively analyzed,and the patients were grouped according to whether they died within 28 d after diagnosis.The clinical characteristics of the two groups were compared.A multivariate analysis of patients'death within 28 d was performed using logistic regression.Results In 53 adult HPS patients,the mortality rate within 28 d was 28.3%(15/53).The survival time of patients was related to white blood cell count(r=0.324,P=0.018),total bilirubin level(r=-0.280,P=0.042)and albumin level(r=0.281,P=0.042),but there was no linear causality(all P>0.05).When compared between the death within 28 d group and the non-death within 28 d group,the differences in patients'age,platelet count,albumin level,creatine kinase isoenzyme level,triacylglycerol level,ferritin level,and central nervous system involvement were statistically significant(all P<0.05).Multivariate logistic regression analysis showed that platelet count<30×109/L,albumin<30 g/L,central nervous system involvement,and ferritin≥10000 ng/ml were independent risk factors for patients'death within 28 d(all P<0.05).Conclusions In adult HPS patients,assessing the risk of early death based on ferritin level,platelet count,albumin level,and neurological symptoms,actively correcting internal environmental disturbances,and enhancing organ support therapy can contribute to survival benefit.

关 键 词：淋巴组织细胞增多症 嗜血细胞性 临床特征 早期死亡 

分 类 号：R55[医药卫生—血液循环系统疾病]