成人显著高铁蛋白血症病因和预后分析及血清铁蛋白在HLH诊断中的价值研究  

Etiological analysis and diagnostic and prognostic value of marked hyper ferritinemia in adults

作　　者：董桂英[1] 刘金涛[2] 张海燕[1] 高伟波[1] 朱继红[1] Dong Gui-ying;Liu Jin-tao;Zhang Hai-yan;Gao Wei-bo;Zhu Ji-hong(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]北京市昌平区中西医结合医院急诊科

出　　处：《中国急救医学》2020年第1期65-69,共5页Chinese Journal of Critical Care Medicine

摘　　要：目的 分析显著高铁蛋白血症的病因分布和疾病预后,探讨血清铁蛋白(serum ferritin,SF)在噬血细胞性淋巴细胞组织增多症(hemophagocytic lymphohistiocytosis,HER)中的诊断意义.方法 纳入2017-01-01至2018-09-30北京大学人民医院门急诊及住院患者中SF≥10 000 μg/L的成人患者140例,根据SF水平分为三组:10 000~19 999 μg/L(A组),20000~29 999 μg/L为B组及≥30 000 μg/L为C组.描述性统计分析患者病因分布,x2检验不同分组HLH诊断比例差异性,Spearman秩相关分析SF水平与预后相关关系.结果 患者SF水平中位数是18 939 μg/L,A组74例(52.9%)、B组及C组均为33例(23.6%).最常见的病因是血液病,其次是结缔组织病(connective tissue disease,CTD).按病因分布的SF水平是不同的.82例(59%)患者仅有一种病因,其他患者均有2种或2种以上病因.HLH 45例,包含CTD继发者14例(30%),其中成人Still病(Adult-onset Still's disease,AOSD)7例(16%)、系统性红斑狼疮(systemic lupus of eryematosus,SLE)6例(14%),其他1例;淋巴瘤继发者11例(25%);EBV感染继发者9例(20%);非病毒感染继发者4例(9%),未能明确原发病者7例(16%).确诊为HLH的患者在A、B和C组中的比例分别为26%、33%和45%,随着SF水平的升高,诊断HLH的比例也随之增加,但通过x2检验进行组间比较发现,组间差异无统计学意义.Spearman秩相关分析SF水平与预后相关系数为0.010,呈正相关,P值为0.906,不具备统计学意义.结论 成人显著高铁蛋白血症病因呈多态性分布,单纯SF显著升高对成人HLH诊断以及对患者预后评估的价值有限.Objective This study aims to analyze the etiological distribution and prognosis of diseases of marked hyper ferritinemia,and to explore the value of ferritin in diagnosis of hemophagocytic lymphohistiocytosis(HLH).Methods A total of 140 patients with ferritin(≥10000μg/L)were enrolled,and they were outpatients or inpatients from the department of emergency of Peking University People's Hospital from Jan 1,2017 to Sep 30,2018.According to the level of serum ferritin,they were further divided into three groups:group A(ferritin:10000-19999μg/L),group B(ferritin:20000-29999μg/L)and group C(femtin>30000μg/L).The pathogenic distribution was analyzed by descriptive statistics method,the difference of proportion of HLH diagnosed in 3 groups was analyzed by x2 test,and the correlation between ferritin level and prognosis was analyzed by Spearman rank correlation analysis.Results The median level of ferritin was 18,939μg/L,74 cases(52.9%)in group A,33 cases(23.6%)in group B and C.The most common cause was hematological diseases,followed by rheumatic immune diseases.The level of ferritin was different according to etiology.There was only one etiology in 82 patients(59%)and two or more etiologies in other patients.There were 45 cases of HLH and 14 cases(30%)was secondary to rheumatic immune system diseases,including 7 cases of Adult-onset S till's disease(16%)and 6 cases of Systemic lupus erythematosus(14%);11 cases was secondary to lymphoma(25%);9 cases was secondary to EBV infection(20%);4 cases was secondary to non-viral infection(9%)and 7 cases of undetermined primary disease(16%).The proportion of patients diagnosed with HLH in group A,group B and group C was 26%,33%and 45%,respectively.With the increase of serum ferritin level,the proportion of patients diagnosed with HLH also increased.However,throug test,the comparison between groups showed that there was no significant difference between groups.Spearman rank correlation analysis showed that the correlation coefficient between ferritin level and prognosis was 0

关 键 词：成人 高铁蛋白血症 病因 噬血细胞性淋巴细胞组织增多症 

分 类 号：R446.11[医药卫生—诊断学]