39例成人继发性噬血细胞综合征实验室检查及临床分析  

Laboratory examination and clinical analysis of 39 cases of adult secondary hemophagocytic syndrome

作　　者：张梦潇 汪英颖[1] 刘亚楠 卢瑞 张爱边 左学兰[1] ZHANG Mengxiao;WANG Yingying;LIU Ya'nan;LU Rui;ZHANG Aibian;ZUO Xuelan(Dept.of Hematology,Zhongnan Hospital of Wuhan University,Wuhan 430071,Hubei,China)

机构地区：[1]武汉大学中南医院血液内科,湖北武汉430071

出　　处：《武汉大学学报（医学版）》2022年第1期128-132,148,共6页Medical Journal of Wuhan University

摘　　要：目的:通过总结成人继发性噬血细胞综合征(HLH)患者的实验室检查、临床特点及预后,以提高对该病的认识。方法:对武汉大学中南医院39例成人继发性HLH患者的病因、临床表现、实验室检查、治疗及预后等进行回顾性分析。结果:39例患者中,血液系统肿瘤15例,单纯感染14例,自身免疫性疾病4例,其它原因6例。临床上以发热(100%)、脾肿大(74%)和肝肿大(38%)为主要表现,实验室检查以血细胞减少(100%)、铁蛋白升高(82%)、高脂血症(54%)、低纤维蛋白原血症(46%)、白细胞介素-6(IL-6)升高(62%)及骨髓噬血现象(64%)为主要特征。随访期内死亡组与存活组患者的实验室结果比较发现,死亡组患者的乳酸脱氢酶(LDH)、C反应蛋白(CRP)和IL-6水平明显高于存活组,差异有统计学意义(P<0.05);Kaplan-Meier生存分析提示多病因组患者总生存率低于单病因组患者(P=0.045),EB病毒(EBV)感染患者总生存率低于未感染EBV者(P=0.036)。结论:成人继发性HLH病因复杂,临床表现具有异质性,对可疑患者及时进行相关实验室检查并结合临床表现有助于早期诊断及治疗。Objective:To summarize the laboratory examination,clinical features,and prognosis of adult secondary hemophagocytic syndrome(HLH)in order to improve the understanding of this disease.Methods:A total of 39 cases of adult secondary HLH in Zhongnan Hospital of Wuhan University were retrospectively analyzed in terms of etiology,clinical manifestations,laboratory examination,treatment,and prognosis.Results:Among the 39 cases,there were 15 cases of hematological neoplasm,14 cases with simple infections,4 cases of autoimmune diseases,and 6 cases of other reasons.The common manifestations included fever(100%),splenomegaly(74%),and hepatomegaly(38%);laboratory tests were characterized by hemocytopenia(100%),elevated ferritin(82%),hyperlipidemia(54%),hypofibrinogenemia(46%),increased interleukin-6(IL-6)(62%),and hemophagocytosis in bone marrow(64%).The comparison of the laboratory results between the death group and the survival group during the follow-up period showed that the levels of serum lactate dehydrogenase(LDH),C-reactive protein(CRP),and IL-6 in the death group were significantly higher than those in the survival group,and the difference was statistically significant(P<0.05).Kaplan-Meier survival analysis showed that the overall survival rate in the multi-etiology group was lower than that in the single-etiology group(P=0.045).The overall survival rate of patients infected with EBV was lower than those without EBV infection(P=0.036).Conclusion:The etiology of adult secondary HLH is complex and the clinical manifestations are heterogeneous.Timely laboratory examination of suspected patients combined with clinical manifestations is helpful for early diagnosis and treatment.

关 键 词：噬血细胞综合征 病因 临床特点 预后 

分 类 号：R55[医药卫生—血液循环系统疾病]