A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by excessive activation of lymphocytes and macrophages, leading to cytokine storm. Infection-associated HLH is most common, and Epstein-Barr virus is the leading triggers. Quick diagnosis is essential for starting the tr...

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Main Authors: Seong-Beom Oh, Chan-Young Koh
Format: Article
Language:English
Published: Korean Society of Pediatric Emergency Medicine 2019-06-01
Series:Pediatric Emergency Medicine Journal
Subjects:
Online Access:http://pemj.org/upload/pdf/pemj-2018-00297.pdf
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author Seong-Beom Oh
Chan-Young Koh
author_facet Seong-Beom Oh
Chan-Young Koh
author_sort Seong-Beom Oh
collection DOAJ
description Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by excessive activation of lymphocytes and macrophages, leading to cytokine storm. Infection-associated HLH is most common, and Epstein-Barr virus is the leading triggers. Quick diagnosis is essential for starting the treatment before irreversible damage. We report a case of 16-year-old boy who presented with unremitted fever, jaundice, and erythematous maculopapular rash all over the body. Investigations showed thrombocytopenia, hyperferritinemia, hypertriglycemia, and the bone marrow biopsy showed hemophagocytosis. Epstein-Barr virus antibody was positive. He responded to chemotherapy as per the HLH-2004 protocol and supportive treatment, and was discharged without complication on day 17.
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spelling doaj.art-d4d69d904c1c4ecdb5fb0e39ec13678b2023-04-04T23:49:01ZengKorean Society of Pediatric Emergency MedicinePediatric Emergency Medicine Journal2383-48972508-55062019-06-0161263010.22470/pemj.2018.0029776A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rashSeong-Beom Oh0Chan-Young Koh1Department of Emergency Medicine, Dankook University College of Medicine, Cheonan, KoreaDepartment of Emergency Medicine, Dankook University College of Medicine, Cheonan, KoreaHemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by excessive activation of lymphocytes and macrophages, leading to cytokine storm. Infection-associated HLH is most common, and Epstein-Barr virus is the leading triggers. Quick diagnosis is essential for starting the treatment before irreversible damage. We report a case of 16-year-old boy who presented with unremitted fever, jaundice, and erythematous maculopapular rash all over the body. Investigations showed thrombocytopenia, hyperferritinemia, hypertriglycemia, and the bone marrow biopsy showed hemophagocytosis. Epstein-Barr virus antibody was positive. He responded to chemotherapy as per the HLH-2004 protocol and supportive treatment, and was discharged without complication on day 17.http://pemj.org/upload/pdf/pemj-2018-00297.pdfepstein-barr virus infectionsexanthemaferritinsfeverlymphohistiocytosis, hemophagocytic
spellingShingle Seong-Beom Oh
Chan-Young Koh
A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash
Pediatric Emergency Medicine Journal
epstein-barr virus infections
exanthema
ferritins
fever
lymphohistiocytosis, hemophagocytic
title A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash
title_full A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash
title_fullStr A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash
title_full_unstemmed A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash
title_short A case of hemophagocytic lymphohistiocytosis caused by an Epstein-Barr virus infection, presenting with unremitting fever and rash
title_sort case of hemophagocytic lymphohistiocytosis caused by an epstein barr virus infection presenting with unremitting fever and rash
topic epstein-barr virus infections
exanthema
ferritins
fever
lymphohistiocytosis, hemophagocytic
url http://pemj.org/upload/pdf/pemj-2018-00297.pdf
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