Hemophagocytic lymphohistiocytosis and Epstein-Barr virus infection in children

Objective. - The authors had for aim to review pediatric cases of EBV-associated hemophagocytic lymphohistiocytosis observed, between January 1999 and December 2007, to compare their characteristics to literature data, and to suggest a pragmatic strategy for diagnosis and treatment. Design. - The following were analyzed for each patient: age at diagnosis, family history, revised Henter diagnostic criteria, EBV serology and/or PCR, results of genetic studies when available, treatment, short and long-term outcome. Results. - Four patients 11 months to seven years of age were admitted for high and prolonged fever, hepato and/or splenomegaly, and biological markers of hemophagocytic lymphohistiocytosis. They were all initially treated with corticosteroids. The outcome was severe for the two younger patients, with acute hepatocellular failure leading to death for the first, and severe neurological impairment for the second. The two older patients responded well to corticosteroids alone, and are alive. There was no recurrence at the end of the study. Conclusions. - Hemophagocytic lymphohistiocytosis is the most severe complication of infectious mononucleosis. A primary cytotoxicity deficiency must be ruled Out, especially in children under two years of age. Treatment is consensus free, but many studies report interesting results in terms of outcome with regimens including etoposide. (C) 2009 Elsevier Masson SAS. All rights reserved.