Fatal Epstein-Barr Virus–Associated Hemophagocytic Syndrome

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 Ep...

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Bibliographic Details
Published in:Blood 1993-12, Vol.82 (11), p.3259-3264
Main Authors: Kikuta, Hideaki, Sakiyama, Yukio, Matsumoto, Shuzo, Oh-lshi, Tsutomu, Nakano, Takashi, Nagashima, Tetsurou, Oka, Toshiaki, Hironaka, Takashi, Hirai, Kanji
Format: Article
Language:English
Subjects:
Base Sequence
Biological and medical sciences
Child
Child, Preschool
DNA, Viral - analysis
Female
Hematologic and hematopoietic diseases
Herpesviridae Infections - microbiology
Herpesviridae Infections - mortality
Herpesvirus 4, Human - genetics
Herpesvirus 4, Human - pathogenicity
Histiocytosis, Non-Langerhans-Cell - microbiology
Histiocytosis, Non-Langerhans-Cell - mortality
Humans
Infant
Male
Medical sciences
Molecular Sequence Data
Other diseases. Hematologic involvement in other diseases
Polymerase Chain Reaction
RNA, Viral - analysis
Tumor Virus Infections
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Description
Summary: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 RNA1 (EBER1) showed that EBER1 was detected in one of two examined liver tissues, which localized in hepatocytes.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood.V82.11.3259.3259