Case Report: Chronic Active Epstein–Barr Virus Infection With Subcutaneous Nodules and Systemic Damage

Chronic active Epstein–Barr virus (CAEBV) infection is a rare and progressive systemic lymphoproliferative disorder often beginning as an infectious mononucleosis (IM)-like illness. It manifests with fever, splenomegaly, hepatitis, lymphadenopathy, and occasionally cytopenias, pneumonitis, and vascu...

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Main Authors: Huating Luo, Zhe Yuan, Bo Qin
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-03-01
Series:Frontiers in Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2022.759834/full
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author Huating Luo
Zhe Yuan
Bo Qin
author_facet Huating Luo
Zhe Yuan
Bo Qin
author_sort Huating Luo
collection DOAJ
description Chronic active Epstein–Barr virus (CAEBV) infection is a rare and progressive systemic lymphoproliferative disorder often beginning as an infectious mononucleosis (IM)-like illness. It manifests with fever, splenomegaly, hepatitis, lymphadenopathy, and occasionally cytopenias, pneumonitis, and vasculitis. We report a 23-year-old woman with fever and subcutaneous nodules first appearing on the limbs and then spreading to the body. Peripheral blood EBV antibodies were elevated and EBV-DNA loads significantly increased. A skin and lymph node biopsy identified T cell-based lymphocyte infiltration and EBV-encoded RNA positivity (EBER+). CAEBV was finally diagnosed. During the illness, her disease progressed to hemophagocytic syndrome (HPS). The patient then successfully received an allogeneic hematopoietic stem cell transplantation (HSCT) at 6 months. Current follow-up at 2 years indicated a stable condition and six negative EBV-DNA tests, and we reviewed the clinical manifestations, mechanism, diagnosis and differential diagnosis, treatment, and prognosis of CAEBV. Finally, subcutaneous nodules may occur when CAEBV invades the skin; therefore, clinicians must identify the cause of these nodules early. HSCT is effective but its timing must be appropriate.
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spelling doaj.art-2e87e71beff04e3bbd0f1e8b50d28e5b2022-12-22T02:41:11ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2022-03-01910.3389/fmed.2022.759834759834Case Report: Chronic Active Epstein–Barr Virus Infection With Subcutaneous Nodules and Systemic DamageHuating LuoZhe YuanBo QinChronic active Epstein–Barr virus (CAEBV) infection is a rare and progressive systemic lymphoproliferative disorder often beginning as an infectious mononucleosis (IM)-like illness. It manifests with fever, splenomegaly, hepatitis, lymphadenopathy, and occasionally cytopenias, pneumonitis, and vasculitis. We report a 23-year-old woman with fever and subcutaneous nodules first appearing on the limbs and then spreading to the body. Peripheral blood EBV antibodies were elevated and EBV-DNA loads significantly increased. A skin and lymph node biopsy identified T cell-based lymphocyte infiltration and EBV-encoded RNA positivity (EBER+). CAEBV was finally diagnosed. During the illness, her disease progressed to hemophagocytic syndrome (HPS). The patient then successfully received an allogeneic hematopoietic stem cell transplantation (HSCT) at 6 months. Current follow-up at 2 years indicated a stable condition and six negative EBV-DNA tests, and we reviewed the clinical manifestations, mechanism, diagnosis and differential diagnosis, treatment, and prognosis of CAEBV. Finally, subcutaneous nodules may occur when CAEBV invades the skin; therefore, clinicians must identify the cause of these nodules early. HSCT is effective but its timing must be appropriate.https://www.frontiersin.org/articles/10.3389/fmed.2022.759834/fullchronic active Epstein-Barr virus infectioncutaneous chronic active EBV infectionEBV-associated hemophagocytic lymphohistiocytosisallogeneic hematopoietic stem cell transplantationEBER
spellingShingle Huating Luo
Zhe Yuan
Bo Qin
Case Report: Chronic Active Epstein–Barr Virus Infection With Subcutaneous Nodules and Systemic Damage
Frontiers in Medicine
chronic active Epstein-Barr virus infection
cutaneous chronic active EBV infection
EBV-associated hemophagocytic lymphohistiocytosis
allogeneic hematopoietic stem cell transplantation
EBER
title Case Report: Chronic Active Epstein–Barr Virus Infection With Subcutaneous Nodules and Systemic Damage
title_full Case Report: Chronic Active Epstein–Barr Virus Infection With Subcutaneous Nodules and Systemic Damage
title_fullStr Case Report: Chronic Active Epstein–Barr Virus Infection With Subcutaneous Nodules and Systemic Damage
title_full_unstemmed Case Report: Chronic Active Epstein–Barr Virus Infection With Subcutaneous Nodules and Systemic Damage
title_short Case Report: Chronic Active Epstein–Barr Virus Infection With Subcutaneous Nodules and Systemic Damage
title_sort case report chronic active epstein barr virus infection with subcutaneous nodules and systemic damage
topic chronic active Epstein-Barr virus infection
cutaneous chronic active EBV infection
EBV-associated hemophagocytic lymphohistiocytosis
allogeneic hematopoietic stem cell transplantation
EBER
url https://www.frontiersin.org/articles/10.3389/fmed.2022.759834/full
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AT zheyuan casereportchronicactiveepsteinbarrvirusinfectionwithsubcutaneousnodulesandsystemicdamage
AT boqin casereportchronicactiveepsteinbarrvirusinfectionwithsubcutaneousnodulesandsystemicdamage