Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis and X-linked lymphoproliferative disease: a mimicker of sepsis in the pediatric intensive care unit.
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 alpha 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.
Mischler
Matthew
M
Division of Pediatric Critical Care Medicine, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan 48109, USA.
Fleming
Geoffrey M
GM
Shanley
Thomas P
TP
Madden
Lisa
L
Levine
John
J
Castle
Valerie
V
Filipovich
Alexandra H
AH
Cornell
Timothy T
TT
D000293:Adolescent; D016026:Bone Marrow Transplantation; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis* / Q000628:therapy; D000068800:Etanercept; D004854:Herpesvirus 4, Human; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use; D015278:Intensive Care Units, Pediatric; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000628:therapy; D008232:Lymphoproliferative Disorders / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use; D018805:Sepsis / Q000175:diagnosis* / Q000628:therapy
D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D000068800:Etanercept