50例嗜血细胞综合征患者的实验室诊断及临床分析  

Laboratory diagnosis and clinical analysis of 50 cases of hemophagocytic syndrome

作　　者：王春玉[1] 彭静[1] 刘为勇[1] 朱耀武[1] 王雄[1] 鲁艳军[1] 孙自镛[1] WANG Chunyu;PENG Jing;LIU Weiyong;ZHU Yaowu;WANG Xiong;LU Yanjun;SUN Ziyong(Department of Clinical Laboratory,Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology,Wuhan,Hubei 430000,China)

机构地区：[1]华中科技大学同济医学院附属同济医院检验科,湖北武汉430000

出　　处：《检验医学与临床》2019年第10期1362-1365,共4页Laboratory Medicine and Clinic

摘　　要：目的分析收集的50例嗜血细胞综合征(HPS)患者的实验室诊断结果及其相关的临床信息,以提高对该类疾病的认识,从而更好地为临床服务。方法对2014年6月至2018年7月该院确诊为HPS的50例患者的实验室检测结果及其临床特点等进行分析。结果 50例HPS患者中,原发性HPS 4例,感染相关HPS 26例,血液相关HPS 15例,自身免疫性疾病3例,原因未明2例。临床表现以发热(100.0%)、肝脾大(74.0%)及淋巴结肿大(56.0%)为主。实验室检查结果以血细胞减少、铁蛋白升高、碱性磷酸酶升高、纤维蛋白原降低、三酰甘油升高、低钠血症等为主。结论 HPS的病因很多,临床表现也多种多样,而对于怀疑HPS的患者需及时、准确地进行相关实验室检查,以达到早期诊断的目的;而该疾病的严重程度与患者是否合并其他相关疾病有关。Objective To analyze the laboratory diagnosis results and related clinical information of 50 patients with hemophagocytic syndrome (HPS) for improving the understanding of the disease and providing better service to the clinic. Methods The laboratory diagnosis results and clinical features of HPS patients were analyzed from June 2014 to July 2018 in Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology. Results Among the 50 patients with HPS,there were 4 cases of primary HPS,26 cases of infection-related diseases,15 cases of hematological diseases,3 cases of autoimmune diseases,and 2 cases of unknown causes.The main clinical manifestations were high fever (100.0%),hepatosplenomegaly (74.0%) and lymphadenopathy (56.0%).The laboratory results mainly showed cytopenia,serum ferritin elevation,alkaline phosphatase elevation,hypofibrinogenemia,hypertriglyceridemia and hyponatremia. Conclusion There are many etiologies and diverse clinical manifestations of HPS.For those patients suspected with HPS,relevant laboratory tests should be conducted timely and accurately to achieve the purpose of early diagnosis.The severity of the disease is consistent with patient′s co-morbidity.

关 键 词：嗜血细胞综合征 实验室检查 病因学 预后 

分 类 号：R551.2[医药卫生—血液循环系统疾病]