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Fatal Epstein-Barr virus-associated hemophagocytic syndrome
H Kikuta, Y Sakiyama, S Matsumoto, T Oh-Ishi, T Nakano, T Nagashima, T Oka, T Hironaka and K Hirai
Department of Pediatrics, Hokkaido University School of Medicine, Sapporo,
Japan.
A virus-associated hemophagocytic syndrome is characterized by high fever,
liver dysfunction, coagulation abnormalities, pancytopenia, and a benign
histiocytic proliferation with prominent hemophagocytosis in bone marrow,
lymph node, spleen, and liver. We describe six Japanese children with fatal
Epstein-Barr virus (EBV)-associated hemophagocytic syndrome. Five of the
six patients had serologic evidence of primary EBV infection at the onset
of their diseases. EBV genomes were detected in all the patients by
Southern blot hybridization or the polymerase chain reaction. Furthermore,
clonality analysis of the EBV genome showed that EBV-infected cells
proliferated monoclonally or biclonally in three examined patients. In situ
hybridization study using EBV- encoded RNA 1 (EBER1) showed that EBER1 was
detected in one of two examined liver tissues, which localized in
hepatocytes.
Volume 82,
Issue 11,
pp. 3259-3264,
12/01/1993
Copyright © 1993 by The American Society of Hematology
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