Pediatr Emerg Med J.  2019 Jun;6(1):26-30. 10.22470/pemj.2018.00297.

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

Affiliations
  • 1Department of Emergency Medicine, Dankook University College of Medicine, Cheonan, Korea. suepunk@naver.com

Abstract

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.

Keyword

Epstein-Barr Virus Infections; Exanthema; Ferritins; Fever; Lymphohistiocytosis, Hemophagocytic

MeSH Terms

Adolescent
Biopsy
Bone Marrow
Diagnosis
Drug Therapy
Epstein-Barr Virus Infections
Exanthema*
Ferritins
Fever*
Herpesvirus 4, Human*
Humans
Jaundice
Lymphocytes
Lymphohistiocytosis, Hemophagocytic*
Macrophages
Male
Thrombocytopenia
Ferritins
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