Journal
MEDICAL AND PEDIATRIC ONCOLOGY
Volume 41, Issue 2, Pages 103-109Publisher
WILEY-LISS
DOI: 10.1002/mpo.10314
Keywords
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis; fatal infectious mononucleosis; fulminant infectious mononucleosis
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Background. Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), also known as EBV-associated hernophagocytic syndrome, develops mostly in children and young adults and may be fatal. Early etoposide treatment has been confirmed to be effective in children. However, it is unclear whether the same treatment is useful in adults. Procedure. To assess whether etoposide is effective in treating young adult cases, we retrospectively studied the therapeutic measures taken and outcomes in 20 young adult cases of EBV-HLH. Eleven cases were registered in our HLH study center in Kyoto and nine derived from the literature. The patients were between 17 and 33 years old and eight were males. The influence of gender, cell lineage (T- or natural killer-), EBV serology pattern, jaundice and treatment on the outcome was assessed. Results and Conclusions. Patients receiving etoposide within four weeks after diagnosis had a good prognosis as five of the seven patients survived compared to one of 13 not treated with etoposide or treated late (chi-square test for survival, P=0.0095). The Kaplan-Meier analysis showed the 2.5-year survival of 85.7+/-13.2% in the early etoposide-treated patients, compared to 10.3+/-9.4% in the remaining patients (log-rank test, P=0.0141). Thus, early etoposide treatment is effective in treating EBV-HLH in young adults as well as in children. (C) 2003 Wiley-Liss, Inc.
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