A case of virus-associated hemophagocytic syndrome / 대한내과학회지

ABSTRACT

Hemophagocytosis refers to the pathologic finding of activated macrophages engulfing erythrocytes, leukocytes, platelets, and their precursor cells. Hemophagocytic syndrome (HS) (more properly referred to as hemophagocytic lymphohistiocytosis) is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly, and hemophagocytosis in the bone marrow, spleen, and lymph nodes. HS is associated with a variety of viral, bacterial, fungal, and parasitic infections, as well as with collagen vascular diseases and malignancies. Epstein-Barr virus (EBV)-associated HS is almost always a fatal disease. The authors present a case of EBV-associated HS. The patient had fever and hepatosplenomegaly and showed pancytopenia, jaundice, and positive EBV viral markers in serum, positive EBV DNA in the liver biopsy specimen, and hemophagocytosis in the bone marrow. The patient died unexpectedly 1 month after admission. We report the details of this case of fatal EBV-associated hemophagocytic syndrome along with a brief review of the literature.