Fatal Hemophagocytic Syndrome : Three Autopsy Cases

Kim, Yi Suk; Kwon, Tae Jung; Chung, Nak Eun; Choi, Young Shik

Abstract

Hemophagocytic Syndrome (HS) is a systemic lymphohistiocytic proliferative disorder associated with infection or malignancies, particularly Epstein-Barr virus (EBV) HS is presented with high fever, skin rash, hepatosplenomegaly, and cytopenia. The characteristic pathologic finding is massive lymphohistiocytic infiltration with hemophagocytosis in various organs including bone marrow, liver, and spleen. Hyperproduction of cytokines by activated T lymphocytes has been presumed to account for the hemophagocytosis and clinical manifestations of HS. We report three childhood autopsy cases with HS which was confirmed by histopathologic examination. According to medical records, all cases had high fever with or without skin rash, cytopenia, AST/ALT elevation, and hyperfibrinogenemia, and showed multiple organ failure eventually. At autopsy, there were no specific gross findings except splenomegaly, but extensive lymphohistiocytic infiltration with hemophagocytosis was seen in various organs including lymph nodes, spleen, liver, and bone marrow. In one of three cases, EBV was identified with in situ hybridization method. As this disease has rapidly progressive clinical course with fatal outcome during childhood, so the possibility of HS should be considered in children presenting with high fever and hepatosplenomegaly.