Published online April 2, 2007
PEDIATRICS Vol. 119 No. 5 May 2007, pp. e1212-e1218 (doi:10.1542/10.1542/peds.2006-1534)

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EXPERIENCE & REASON

Epstein-Barr Virus–Induced Hemophagocytic Lymphohistiocytosis and X-Linked Lymphoproliferative Disease: A Mimicker of Sepsis in the Pediatric Intensive Care Unit

Matthew Mischler, MD^a, Geoffrey M. Fleming, MD^a, Thomas P. Shanley, MD^a, Lisa Madden, MD^b, John Levine, MD^b, Valerie Castle, MD^b, Alexandra H. Filipovich, MD^c and Timothy T. Cornell, MD^a

^a Divisions of Critical Care Medicine
^b Hematology and Oncology, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan
^c Bone Marrow Transplantation, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio

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 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.

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Key Words: X-linked lymphoproliferative disease • hemophagocytic lymphohistiocytosis • systemic inflammatory response syndrome • infectious mononucleosis • sepsis • Epstein-Barr virus • Etanercept

Abbreviations: EBV, Epstein-Barr virus • HLH, hemophagocytic lymphohistiocytosis • XLP, X-linked lymphoproliferative • TNF-, tumor necrosis factor • IL, interleukin • SAP, signaling lymphocyte activation molecule–associated protein • Th, T helper • LMP-1, latent membrane protein 1 • IVIG, intravenous immunoglobulin

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Accepted Oct 31, 2006.

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