Evaluation of Clinical Characteristics and Prognostic Factors in the Epstein-Barr Virus-Associated Hemophagocytic Syndrome

Park, Sae Myung; Park, Song Hee; Lyu, Chuhl Joo; Yang, Chang Hyun; Oh, Seung Hwan; Kim, Kir Young

Abstract

BACKGROUND: A virus-associated hemophagocytic syndrome(VAHS) is a non-neoplastic, generalized histiocytic proliferation with prominent hemophagocytosis associated with a systemic viral infection. Although VAHS is generally a benign process, Epstein-Barr virus- associated hemophagocytic syndrome(EBV-AHS) is often fatal and has a high mortality rate. Therefore, clinical and laboratory findings, virology studies, treatment and prognosis in EBV- AHS are reviewed.
METHODS
We retrospectively analyzed various clinical datas of 7 patients who were diagnosed as EBV-AHS by EBV panel test, mRNA in situ hybridization(1 case) and diagnostic guidelines of Histiocyte Society and followed up to the Department of Pediatrics, Severance Hospital, Yonsei University Medical College from January 1990 to December 1997.
RESULTS
The mean age at diagnosis was 2.8 years(range; 0.8~5.4). EBV-AHS was caused by reactivation of EBV in 6 cases and primary infection in 1 case. Persistent fever and hepatosplenomegaly were observed in all cases. Hyperferritinemia was detected in 5 cases. Hypertriglyceridemia was noted in 4 cases among 5 cases tested and hypofibrinogenemia was detected in 5 cases among 5 cases tested. Benign histiocytic infiltration(at least above 5% of all nucleated marrow cells), hemophagocytosis and normocellularity in bone marrow were observed in all cases. The mortality rate was 57%, the main causes of death were bleeding and sepsis. In analysis of prognostic factors associated with death, there was a correlation between hypofibrinogenemia, hepatomegaly, hypoalbuminemia and death, although insignificant statistically.
CONCLUSION
In our study, EBV-AHS was caused by reactivation of EBV in most cases. The mortality rate was 57% and the main causes of death were bleeding and sepsis. In analysis of prognostic factors associated with death, there was a correlation between hypofibrinogenemia, hepatomegaly and hypoalbuminemia to a certain degree. But, further study of larger numbers of patients and longer follow-up is needed to clarify the prognostic factors.