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

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.