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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Frontiers Media S.A.
2022-03-01
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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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language | English |
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publishDate | 2022-03-01 |
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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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