A Case of Epstein-Barr Virus Associated Hemophagocytic Syndrome During Pregnancy.

Lee, Jae Ho; Gu, Bon Sang; Lee, Ki Rok; Lee, Jae Sung; Yang, Hee Dong; Song, In Chul

Case Report

Korean Journal of Obstetrics & Gynecology 1999;42(8):1844-1848.
Published online January 1, 2001.

A Case of Epstein-Barr Virus Associated Hemophagocytic Syndrome During Pregnancy.

Jae Ho Lee, Bon Sang Gu, Ki Rok Lee, Jae Sung Lee, Hee Dong Yang, In Chul Song

Abstract

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. Three phases of disease progression can be defined. In the first week, there is mild leukocytosis and myeloid hyperplasia in the marrow and the fever is resistant to antipyretics and antibiotics. In the second phase, usually corresponding to the 2nd to 3rd week of the illness, the fever persists and jaundice and hepatosplenomegaly may develop and the marrow now reveals the presence of atypical or transformed T lymphocytes and a scattering of histiocytes with hemophagocytosis. In the third phase, the disease progresses to a full-blown hemophagocytic syndrome with coagulopathy and lung infiltrates. The marrow in this stage is usually hypoplastic with florid histiocytic proliferation and hemophagocytosis and the patients usually die within 1-2 months. We describe a pregnant woman with fatal hemophagocytic syndrome. Virologic study strongly suggests that Epstein-Barr virus implicated in the pathogenesis of this patient.

Key Words: Hemophagocytic syndrome, Virus-associated hemophagocytic syndrome, Epstein-Barr virus associated hemophagocytic syndrome, Epstein-Barr