Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis and X-linked lymphoproliferative disease: A mimicker of sepsis in the Pediatric Intensive Care Unit

Matthew Mischler, Geoffrey M. Fleming, Thomas P. Shanley, Lisa Madden, John Levine, Valerie Castle, Alexandra H. Filipovich, Timothy T. Cornell*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

24 Scopus citations

Abstract

A rare complication of infection with the Epstein-Barr virus is the development of hemophagocytic lymphohistiocytosis. Although most cases of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis develop in immunocompetent individuals, the rare immunodeficiency X-linked lymphoproliferative disease is often unmasked by Epstein-Barr virus infection and is clinically indistinguishable from Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis. We describe the clinical course and management of a previously healthy 17-year-old boy who presented with hemodynamic collapse and severe systemic inflammatory response syndrome resulting from overwhelming hemophagocytosis in the setting of X-linked lymphoproliferative disease. A novel therapeutic approach using anti-tumor necrosis factor α therapy was instituted, aimed at attenuating the viral-induced hyperinflammatory state. Given the similarity to overwhelming sepsis, yet a substantially different therapeutic approach, this case illustrates the importance of early recognition and prompt treatment that are necessary to reduce the high morbidity and mortality associated with Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis and X-linked lymphoproliferative disease.

Original languageEnglish (US)
Pages (from-to)e1212-e1218
JournalPediatrics
Volume119
Issue number5
DOIs
StatePublished - May 2007

Keywords

  • Epstein-Barr virus
  • Etanercept
  • Hemophagocytic lymphohistiocytosis
  • Infectious mononucleosis
  • Sepsis
  • Systemic inflammatory response syndrome
  • X-linked lymphoproliferative disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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