EBV相关噬血细胞综合征临床特征和预后分析
作者:
作者单位:

作者简介:

通讯作者:

中图分类号:

R551

基金项目:

江苏省青年医学重点人才(QNRC2016791);江苏省自然科学基金(BK20171181);江苏省科技厅社会发展重点项目(BE2019638);江苏省自然科学基金(BK2017025)


Clinical features and prognosis analysis of EBV ⁃ associated hemophagocytic lymphohistiocytosis
Author:
Affiliation:

Fund Project:

  • 摘要
  • |
  • 图/表
  • |
  • 访问统计
  • |
  • 参考文献
  • |
  • 相似文献
  • |
  • 引证文献
  • |
  • 资源附件
  • |
  • 文章评论
    摘要:

    目的:基于多中心数据回顾性分析EB病毒(Epstein-Barr virus,EBV)相关性噬血细胞综合征(hemophagocytic lym- phohistiocytosis,EBV-HLH)患者的临床特点及预后危险因素。方法:选取2013年1月—2020年8月淮海淋巴瘤协作组中9个医疗中心的132例EBV-HLH患者,收集其临床病理资料。使用限制立方样条计算EBV -DNA拷贝数的最佳截断值;采用Cox 比例风险模型进行单因素和多因素分析;绘制患者的Kaplan -Meie r曲线;组间比较采用Log-rank检验。结果:132例患者的中位年龄为25岁,其中儿童(<14岁)61例(46.21%),老年患者(≥60岁)25例(18.94%),发热患者124例(93.94%),血清铁蛋白增高116例(87.88%)。儿童患者的预后情况优于成人(P<0.001),两组患者的半年累积生存率分别为90.2%和38.0%。儿童组患者白蛋白、血清甘油三酯、谷丙转氨酶、淋巴细胞百分比高于成人组患者,而血肌酐低于成人组。存活组的年龄、血肌酐低于死亡组,而血清甘油三酯高于死亡组(P<0.05)。多因素分析显示年龄和EBV-DNA拷贝数是影响EBV-HLH患者生存结局的独立危险因素。结论:EBV -HLH早期病死率高,预后差,初诊EBV-DNA拷贝数和年龄是影响EBV-HLH患者预后的不良因素。

    Abstract:

    Objective:To retrospectively analyze the clinical characteristics and prognostic risk factors of EBV-associated hemophagocytic syndrome based on multicenter data.Methods:A total of 132 patients with EBV-HLH from 9 medical centers in Huaihai Lymphoma Working Group from January 2013 to August 2020 were selected and their clinicopathological data were collected. The optimal cut-off point of EBV-DNA copy number was calculated using restricted cubic spline. Cox proportional hazards model was used for univariate and multivariate analysis. Kaplan-Meier curves of patients were drawn. Log-rank test was used for comparison between groups. Results:The median age of 132 patients was 25 years old,including 61 children(< 14 years old)(46.21%),25 elderly patients(≥60 years old)(18.94%),124 patients(93.94%)with fever,and 116 patients(87.88%)with elevated serum ferritin. The prognosis of children patients was better than that of adults(P < 0.001),and the six -month cumulative survival rates of the two groups were 90.2% and 38.0%,respectively. The percentage of albumin,serum triglyceride,alanine aminotransferase and lymphocyte in the children group was higher than that in the adult group,while the serum creatinine was lower than that in the adult group. The age and serum creatinine of the survival group were lower than those of the death group,while the levels of serum triglyceride were higher than those of the death group(P < 0.05). Multivariable analysis showed that age and EBV-DNA copy number were independent risk factors for survival outcome of EBV-HLH patients. Conclusion:The early mortality of EBV-HLH is high and the prognosis is poor. The EBV-DNA copy number and age at initial diagnosis are the adverse factors affecting the prognosis of EBV-HLH patients.

    参考文献
    相似文献
    引证文献
引用本文

唐旭东,胡灵灵,沈子园,张颢,苗雨青,王春玲,王玲,叶静静,顾伟英,朱太岗,方拥军,桑威,赵世鸿. EBV相关噬血细胞综合征临床特征和预后分析[J].南京医科大学学报(自然科学版),2022,42(12):1697-1702,1709

复制
分享
文章指标
  • 点击次数:
  • 下载次数:
  • HTML阅读次数:
  • 引用次数:
历史
  • 收稿日期:
  • 最后修改日期:
  • 录用日期:
  • 在线发布日期: 2022-12-12
  • 出版日期:
通知关闭
郑重声明