|Table of Contents|

Clinical features and prognosis of Epstein-Barr virus associated hemophagocytic syndrome

Journal Of Modern Oncology[ISSN:1672-4992/CN:61-1415/R]

Issue:
2023 20
Page:
3840-3843
Research Field:
Publishing date:

Info

Title:
Clinical features and prognosis of Epstein-Barr virus associated hemophagocytic syndrome
Author(s):
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.
Keywords:
Epstein-Barr virus; infection; hemophagocytic syndrome; clinical features; prognosis
PACS:
R733
DOI:
10.3969/j.issn.1672-4992.2023.20.023
Abstract:
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.

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