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

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Mischler, M. Articles by Cornell, T. T. Search for Related Content PubMed PubMed Citation Articles by Mischler, M. Articles by Cornell, T. T. Related Collections Infectious Disease & Immunity Social Bookmarking                         What's this?

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.

---

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

---

Accepted Oct 31, 2006.

CiteULike    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				M. L. Gulley and W. Tang Laboratory Assays for Epstein-Barr Virus-Related Disease J. Mol. Diagn., July 1, 2008; 10(4): 279 - 292. [Abstract] [Full Text] [PDF]