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

Research output: Contribution to journalArticlepeer-review

29 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
Pages (from-to)e1212-e1218
JournalPediatrics
Volume119
Issue number5
DOIs
StatePublished - May 2007
Externally publishedYes

Keywords

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

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