文献检索文档翻译深度研究
Suppr Zotero 插件Zotero 插件
邀请有礼套餐&价格历史记录

新学期,新优惠

限时优惠:9月1日-9月22日

30天高级会员仅需29元

1天体验卡首发特惠仅需5.99元

了解详情
不再提醒
插件&应用
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
高级版
套餐订阅购买积分包
AI 工具
文献检索文档翻译深度研究
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2025

成人原发性 EBV 感染致噬血细胞性淋巴组织细胞增生症自发缓解

Spontaneous Recovery of Hemophagocytic Lymphohistiocytosis Due to Primary Epstein-Barr Virus Infection in an Adult Patient.

机构信息

Department of Internal Medicine, Tokyo Bay Urayasu Ichikawa Medical Center, Urayasu, Chiba, Japan.

Department of Rheumatology, Tokyo Bay Urayasu Ichikawa Medical Center, Urayasu, Chiba, Japan.

出版信息

Am J Case Rep. 2021 Oct 17;22:e933272. doi: 10.12659/AJCR.933272.


DOI:10.12659/AJCR.933272
PMID:34657119
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8532072/
Abstract

BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening systemic hyperinflammatory condition. Most adult HLH cases are secondary to infection, malignancy, and rheumatic diseases. Epstein-Barr virus (EBV) infection is the most frequent cause of infection-induced HLH. Early treatment with dexamethasone, etoposide, and cyclosporine is generally recommended for adult patients with HLH. However, this treatment regimen was established based on pediatric clinical trial data; thus, its efficacy and validity in adults remain unclear. Because little is known about the disease course of untreated adult EBV-associated HLH (EBV-HLH), we report a case of an adult patient who recovered from EBV-HLH spontaneously without specific treatment and discuss potential treatment strategies. CASE REPORT A 34-year-old man presented to the emergency department with a 7-day history of fever, headache, and sore throat. The main laboratory test abnormalities were elevated liver enzymes, hyperbilirubinemia, hypertriglyceridemia, and hyperferritinemia. Serologic tests confirmed acute primary EBV infection. He was diagnosed with EBV-HLH based on the HLH-2004 diagnostic criteria and the HLH probability calculator (HScore). Because he was clinically stable, we did not initiate immunosuppressive/cytotoxic treatment targeting HLH. High-grade fever persisted, but the abnormalities in his laboratory data improved spontaneously, and he did not develop major organ failure. His fever resolved on day 29 without HLH-specific treatment. CONCLUSIONS In clinically stable adult patients with EBV-HLH without major organ failure, it might be an acceptable alternative to observe the patient for several weeks before initiating HLH-specific treatment. Further research is required to better predict the subset of patients who can be safely observed without treatment.

摘要

背景:噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的危及生命的全身性炎症反应过度综合征。大多数成人 HLH 病例继发于感染、恶性肿瘤和风湿性疾病。EB 病毒(EBV)感染是感染相关性 HLH 最常见的病因。一般建议对成人 HLH 患者早期采用地塞米松、依托泊苷和环孢素治疗。然而,该治疗方案是基于儿科临床试验数据建立的;因此,其在成人中的疗效和有效性尚不清楚。由于成人未经治疗的 EBV 相关性 HLH(EBV-HLH)的疾病过程知之甚少,我们报告了一例成人患者在未接受特定治疗的情况下自发从 EBV-HLH 中康复,并讨论了潜在的治疗策略。

病例报告:一名 34 岁男性因发热、头痛和咽痛 7 天就诊于急诊。主要实验室检查异常包括肝酶升高、高胆红素血症、高甘油三酯血症和高铁蛋白血症。血清学检查证实为急性原发性 EBV 感染。根据 HLH-2004 诊断标准和 HLH 概率计算器(HScore),他被诊断为 EBV-HLH。由于他临床稳定,我们没有启动针对 HLH 的免疫抑制/细胞毒性治疗。高热持续存在,但实验室数据异常自发改善,他没有发生主要器官衰竭。他的发热在第 29 天自行消退,没有接受 HLH 特异性治疗。

结论:在没有主要器官衰竭的临床稳定的 EBV-HLH 成人患者中,在启动 HLH 特异性治疗前观察患者数周可能是一种可以接受的替代方案。需要进一步研究以更好地预测可以安全观察而无需治疗的患者亚组。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/041f/8532072/89530761ded6/amjcaserep-22-e933272-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/041f/8532072/5489655cac7b/amjcaserep-22-e933272-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/041f/8532072/48d341a13b48/amjcaserep-22-e933272-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/041f/8532072/89530761ded6/amjcaserep-22-e933272-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/041f/8532072/5489655cac7b/amjcaserep-22-e933272-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/041f/8532072/48d341a13b48/amjcaserep-22-e933272-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/041f/8532072/89530761ded6/amjcaserep-22-e933272-g003.jpg

相似文献

[1]
Spontaneous Recovery of Hemophagocytic Lymphohistiocytosis Due to Primary Epstein-Barr Virus Infection in an Adult Patient.

Am J Case Rep. 2021-10-17

[2]
Prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children: report of the Japan Histiocytosis Study Group.

Pediatr Blood Cancer. 2014-2-17

[3]
Epstein-Barr virus-related hemophagocytic lymphohistiocytosis complicated with coronary artery dilation and acute renal injury in a boy with a novel X-linked inhibitor of apoptosis protein (XIAP) variant: a case report.

BMC Pediatr. 2020-10-2

[4]
A case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with severe cardiac complications.

BMC Pediatr. 2016-10-28

[5]
[Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis during pregnancy].

Rinsho Ketsueki. 2017

[6]
Improved hemophagocytic lymphohistiocytosis index predicts prognosis of adult Epstein-Barr virus-associated HLH patients.

Ann Med. 2023-12

[7]
Epstein-Barr virus central nervous system involvement in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

Ann Hematol. 2022-11

[8]
Clinical laboratory Analysis of EB Virus Associated Hemophagocytic Lymphohistiocytosis in Children.

Altern Ther Health Med. 2024-5

[9]
Virus-triggered secondary hemophagocytic lymphohistiocytosis.

Acta Paediatr. 2021-10

[10]
[Clinical analysis and follow-up study of Epstein-Barr virus associated-hemophagocytic lymphohistiocytosis in childhood].

Zhonghua Er Ke Za Zhi. 2010-2

引用本文的文献

[1]
Predictive model for early death risk in pediatric hemophagocytic lymphohistiocytosis patients based on machine learning.

Heliyon. 2023-11-11

[2]
Plasma Exosomal Proteomic Pattern of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis.

Front Microbiol. 2022-4-6

本文引用的文献

[1]
Haemophagocytic lymphohistiocytosis that spontaneously resolved: a case of EBV.

BMJ Case Rep. 2020-11-12

[2]
Hemophagocytic lymphohistiocytosis: An update on pathogenesis, diagnosis, and therapy.

Best Pract Res Clin Rheumatol. 2020-8

[3]
Emapalumab in Children with Primary Hemophagocytic Lymphohistiocytosis.

N Engl J Med. 2020-5-7

[4]
Ruxolitinib in adult patients with secondary haemophagocytic lymphohistiocytosis: an open-label, single-centre, pilot trial.

Lancet Haematol. 2019-12

[5]
Recommendations for the management of hemophagocytic lymphohistiocytosis in adults.

Blood. 2019-4-16

[6]
Adult onset haemophagocytic lymphohistiocytosis prognosis is affected by underlying disease: analysis of a single-institution series of 174 patients.

Swiss Med Wkly. 2018-9-23

[7]
Epstein-Barr Virus Hepatitis: A Review of Clinicopathologic Features and Differential Diagnosis.

Arch Pathol Lab Med. 2018-10

[8]
Treatment outcomes and prognostic factors in adult patients with secondary hemophagocytic lymphohistiocytosis not associated with malignancy.

Haematologica. 2018-9-13

[9]
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults and adolescents-a life-threatening disease: analysis of 133 cases from a single center.

Hematology. 2018-12

[10]
Epstein-Barr Virus-associated Hemophagocytic Lymphohistiocytosis in Adults: A Retrospective Analysis of 23 Patients in China.

Isr Med Assoc J. 2018-2

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

推荐工具

医学文档翻译智能文献检索