Case of Chronic Active Epstein-Barr Virus Infection Developed Hemophagocytic Lymphohistiocytosis after COVID-19 Infection

In children, prolonged primary Epstein-Barr virus (EBV) infection or reactivation may lead to the development of T or natural killer cell lymphoproliferative disorders, classified as chronic active EBV infection (CAEBV). CAEBV is a prolonged systemic illness lasting beyond three months, with elevate...

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Main Authors: Sumin Jo, Hye Lim Jung, Sooyeon Lim, Eun Sil Kim, Aram Yang, Ji Hee Kwak, Deok Soo Kim, Jung Yeon Shim, Jae Won Shim
Format: Article
Language:English
Published: The Korean Society of Pediatric Hematology-Oncology 2023-10-01
Series:Clinical Pediatric Hematology-Oncology
Subjects:
Online Access:https://doi.org/10.15264/cpho.2023.30.2.80
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author Sumin Jo
Hye Lim Jung
Sooyeon Lim
Eun Sil Kim
Aram Yang
Ji Hee Kwak
Deok Soo Kim
Jung Yeon Shim
Jae Won Shim
author_facet Sumin Jo
Hye Lim Jung
Sooyeon Lim
Eun Sil Kim
Aram Yang
Ji Hee Kwak
Deok Soo Kim
Jung Yeon Shim
Jae Won Shim
author_sort Sumin Jo
collection DOAJ
description In children, prolonged primary Epstein-Barr virus (EBV) infection or reactivation may lead to the development of T or natural killer cell lymphoproliferative disorders, classified as chronic active EBV infection (CAEBV). CAEBV is a prolonged systemic illness lasting beyond three months, with elevated EBV DNA levels in the serum or affected tissue and a lack of underlying immunodeficiency. Notably, EBV is the most common virus that induces secondary hemophagocytic lymphohistiocytosis (HLH), and Coronavirus disease 2019 (COVID-19) has been reported to trigger HLH. We present the case of a 20-year-old Korean male who was diagnosed with CAEBV and developed HLH after being infected with COVID-19. The patient met 7 out of 8 criteria for HLH. Following treatment with corticosteroids and intravenous immunoglobulin, the patient recovered from HLH without additional chemotherapy. HLH treatment should be tailored to each patient based on clinical presentation and disease severity. Prolonged and regular evaluation for developing HLH or lymphoma is necessary for patients with CAEBV.
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spelling doaj.art-e5084756b8114e5dae82633b2ddf86192023-11-02T02:40:58ZengThe Korean Society of Pediatric Hematology-OncologyClinical Pediatric Hematology-Oncology2233-52502023-10-01302808410.15264/cpho.2023.30.2.80cpho.2023.30.2.80Case of Chronic Active Epstein-Barr Virus Infection Developed Hemophagocytic Lymphohistiocytosis after COVID-19 InfectionSumin Jo0Hye Lim Jung1Sooyeon Lim2Eun Sil Kim3Aram Yang4Ji Hee Kwak5Deok Soo Kim6Jung Yeon Shim7Jae Won Shim8Department of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaIn children, prolonged primary Epstein-Barr virus (EBV) infection or reactivation may lead to the development of T or natural killer cell lymphoproliferative disorders, classified as chronic active EBV infection (CAEBV). CAEBV is a prolonged systemic illness lasting beyond three months, with elevated EBV DNA levels in the serum or affected tissue and a lack of underlying immunodeficiency. Notably, EBV is the most common virus that induces secondary hemophagocytic lymphohistiocytosis (HLH), and Coronavirus disease 2019 (COVID-19) has been reported to trigger HLH. We present the case of a 20-year-old Korean male who was diagnosed with CAEBV and developed HLH after being infected with COVID-19. The patient met 7 out of 8 criteria for HLH. Following treatment with corticosteroids and intravenous immunoglobulin, the patient recovered from HLH without additional chemotherapy. HLH treatment should be tailored to each patient based on clinical presentation and disease severity. Prolonged and regular evaluation for developing HLH or lymphoma is necessary for patients with CAEBV.https://doi.org/10.15264/cpho.2023.30.2.80epstein-barr virus infectionschronic active epstein-barr virus infectionhemophagocytic lymphohistiocytosiscovid-19
spellingShingle Sumin Jo
Hye Lim Jung
Sooyeon Lim
Eun Sil Kim
Aram Yang
Ji Hee Kwak
Deok Soo Kim
Jung Yeon Shim
Jae Won Shim
Case of Chronic Active Epstein-Barr Virus Infection Developed Hemophagocytic Lymphohistiocytosis after COVID-19 Infection
Clinical Pediatric Hematology-Oncology
epstein-barr virus infections
chronic active epstein-barr virus infection
hemophagocytic lymphohistiocytosis
covid-19
title Case of Chronic Active Epstein-Barr Virus Infection Developed Hemophagocytic Lymphohistiocytosis after COVID-19 Infection
title_full Case of Chronic Active Epstein-Barr Virus Infection Developed Hemophagocytic Lymphohistiocytosis after COVID-19 Infection
title_fullStr Case of Chronic Active Epstein-Barr Virus Infection Developed Hemophagocytic Lymphohistiocytosis after COVID-19 Infection
title_full_unstemmed Case of Chronic Active Epstein-Barr Virus Infection Developed Hemophagocytic Lymphohistiocytosis after COVID-19 Infection
title_short Case of Chronic Active Epstein-Barr Virus Infection Developed Hemophagocytic Lymphohistiocytosis after COVID-19 Infection
title_sort case of chronic active epstein barr virus infection developed hemophagocytic lymphohistiocytosis after covid 19 infection
topic epstein-barr virus infections
chronic active epstein-barr virus infection
hemophagocytic lymphohistiocytosis
covid-19
url https://doi.org/10.15264/cpho.2023.30.2.80
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