59例成人EB病毒相关噬血细胞综合征临床特点和预后分析

目的:分析成人EB病毒相关噬血细胞综合征(EBV-HLH)患者的临床特点和预后危险因素,提高对该疾病的认识和诊治水平。方法:对2013年1月至2018年8月间南昌大学第一附属医院诊治的EBV-HLH 59例成人患者的临床特点和生存资料进行回顾性综合分析。结果:59例患者中以发热(100%)、肝功能异常(91.5%)最常见,皮疹(1.7%)、神经系统症状(3.4%)少见;血清铁蛋白升高(96.6%)、乳酸脱氢酶(LDH)升高(96.6%)、白蛋白(Alb)下降(93.2%)及可溶性CD25(s CD25)水平≥2400 U/ml(92.3%)均比较常见。本组患者中位生存时间为2.5±0.7个月,1、3、6、12个月总体生存率分别是69.5%±6.0%、44.7%±6.6%、23.9%±5.8%和19.7%±5.5%。生存资料单因素分析显示,初诊时EBV-DNA拷贝数≥5×10^5拷贝数/ml(P=0.028)、LDH水平≥600 U/L(P=0.012)、Plt计数<20×10^9/L(P=0.022)的患者预后更差,系统使用HLH-94/2004方案治疗能有效改善EBV-HLH患者预后(P<0.001)。多因素分析显示,LDH水平≥600 U/L(P=0.01)、Plt计数<20×1~09/L(P=0.013)、未系统使用HLH-94/2004方案治疗(P<0.001)均是影响患者生存时间的独立预后不良因素。结论:EBV-HLH患者病情凶险,早期死亡率高,初诊时EBV-DNA拷贝数≥5×10^5/ml、LDH水平≥600 U/L、Plt计数<20×10^9/L的患者预后不良,HLH-94/2004方案治疗能有效改善患者生存情况,应尽早系统治疗。

Clinical Characteristics and Prognostic Analysis of 59 Adult Patients with Epstein-Barr Virus-Associated Hemophagocytic Syndrome

Objective:To analyze the clinical characteristics and the prognostic risk factors of adult patients with Epestein-Barr virus-associated hemophagocytic syndrome(EBV-HLH)so as to enhance the understanding of EBV-HLH and diagnosis and treatment level.Methods:The clinical manifestation and survival data of 59 adult patients with EBVHLH admitted in our hospital from January 2013 to August 2018 were analyzed retrospectively.Results:The most common clinical manifestations of 59 patients were high fever(100%),liver dysfunction(91.5%),however the skin rashes(1.7%),and neurologic abnormality(3.4%)were rare.96.6%of the patients showed the elevation of serum ferritin and LDH level,and hypoproteinemia and sCD25≥2400 U/ml were found in 93.2%and 92.3%of the patients,respectively.The median survival time of 59 patients was 2.5±0.7 months;overall survival rate of 1,3,6 and 12-month was 69.5%±6.0%,44.7%±6.6%,23.9%±5.8%,19.7%±5.5%,respectively.Univariate survival analysis showed that the patients with EBV-DNA copies≥5×105/ml(P<0.05),LDH level≥600 U/L(P<0.05)and Plt count<20×10^9/L(P<0.05)had poor prognosis,and there was statistically difference in the overall survival rate(P<0.01)between HLH-94/2004 group and the group treated without etoposide(not HLH-94/2004).Multivariate analysis revealed that LDH level≥600 U/L(P<0.05),Plt count<20×10^9/L(P<0.05)and treatment protocol(not HLH-94/2004)(P<0.01)were independent prognostic risk factors in 59 patients with EBV-HLH.Conclusions:EBV-HLH assocites with severe clinical features,high mortality rate and poor prognosis of patients.EBV-DNA copies≥5×10^5/ml(P<0.05),LDH level≥600 U/L(P<0.05)and Plt count<20×10^9/L(P<0.05)are the poor prognostic factors,and the treatment with HLH-94/2004 protocol can effectively improve the survival of EBV-HLH patients,should be applied as early as possible.