摘要
目的:分析EB病毒相关噬血细胞综合征(Epstein-Barr virus associated hemophagocytic lymphohistiocytosis,EBV-HLH)患者的临床特征和预后因素,提高对该病的认识和诊治水平。方法:回顾性分析本中心诊治的41例EBV-HLH患者的临床特征和生存资料。结果:41例患者无明显性别差异,中位年龄53(15~77)岁。单纯EBV感染27例。所有患者以发热、脾肿大、呼吸系统症状为主,血清LDH、sCD25、铁蛋白、炎症指标均升高、骨髓噬血现象比较常见,严重血小板减少及凝血障碍少见。中位生存时间为4(1.3~8)个月,1、3、6、12个月总生存(OS)率分别为73.1%、58.5%、39.0%、21.9%。接受HLH-94/2004方案治疗的患者预后更好(P=0.031)。单因素分析显示,初诊LDH水平≥600 U/L、PCT≥1 ng/mL、纤维蛋白原<1.5 g/L、未系统接受HLH-94/2004方案治疗的患者预后更差。多因素分析显示,血浆纤维蛋白原<1.5 g/L及未系统接受HLH-94/2004方案治疗是影响患者OS的独立危险因素。结论:EBV-HLH患者早期死亡率高,初诊时LDH水平≥600 U/L、PCT≥1 ng/mL、纤维蛋白原<1.5 g/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。
Objective:To analyze the clinical characteristics and prognostic factors of patients with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis(EBV-HLH),and to improve the understanding,diagnosis and treatment of EBV-HLH.Methods:The clinical characteristics and survival data of 41 patients with EBV-HLH diagnosed and treated in our center were retrospectively analyzed.Results:There was no significant gender difference in 41 patients,with a median age of 53(15~77)years old.Simple EBV infection was observed in 27 cases.All patients presented with fever,splenomegaly and respiratory symptoms.The serum LDH,sCD25,ferritin and inflammatory indicators were elevated,bone marrow hemophagy was common,and severe thrombocytopenia and coagulopathy were rare.The median survival time was 4(1.3~8)months,and the overall survival(OS)rates at 1,3,6 and 12 months were 73.1%,58.5%,39.0%and 21.9%,respectively.Patients treated with HLH-94/2004 had better outcomes(P=0.031).Univariate analysis showed that patients with initial LDH level≥600 U/L,PCT≥1 ng/mL,fibrinogen<1.5 g/L and who did not receive HLH-94/2004 regimen had worse prognosis.Multivariate analysis showed that plasma fibrinogen<1.5 g/L and failure to systematically receive HLH-94/2004 regimen were independent risk factors affecting OS.Conclusion:EBV-HLH patients have high early mortality,and patients with LDH level≥600 U/L,PCT≥1 ng/mL and fibrinogen<1.5 g/L at initial diagnosis have poor prognosis.HLH-94/2004 regimen can effectively improve the survival of patients,so systematic treatment should be given as soon as possible.
作者
王亚茹
贺建霞
耿晔
白斯君
史殷雪
高轶男
张彩霞
WANG Yaru;HE Jianxia;GENG Ye;BAI Sijun;SHI Yinxue;GAO Yinan;ZHANG Caixia(Department of Hematology,Shanxi Provincial People's Hospital,Shanxi Taiyuan 030012,China)
出处
《现代肿瘤医学》
CAS
北大核心
2023年第20期3840-3843,共4页
Journal of Modern Oncology
关键词
EB病毒
感染
噬血细胞综合征
临床特征
预后
Epstein-Barr virus
infection
hemophagocytic syndrome
clinical features
prognosis
