Abstract
Hemophagocytic syndrome (HPS) is a clinicopathologic entity characterized by systemic proliferation of benign hemophagocytic histiocytes in the bone marrow, associated with fever, cytopenias, liver dysfunction and coagulopathy. We report a case of HPS associated with cytomegalovirus (CMV) infection in a patient with rheumatoid arthritis (RA). A 75 year-old female, who had been diagnosed with RA 15 years previously, was admitted to our hospital for the operation of atlantoaxial subluxation. She suddenly had a high fever and a severe diarrhea, her peripheral blood parameters showed pancytopenia and bone marrow aspirate revealed a decreased nuclear cell count and macrophage phagocytizing blood cells indicating HPS. To take into consideration her clinical course, complicating with heart failure, acute renal failure, liver dysfunction and DIC, her HPS was classified considered to be a severe form of HPS. We administered 1, 000mg of methylprednisolone for 3 days followed by 60mg of prednisolone (PSL) daily, but her condition did not improved. Then we administered etoposide (50mg·day-1) in addition to PSL. Three days after the initiation of etoposide injection, she died from acute pulmonary thromboembolism revealed by postmortem examination. We speculated the etiology for the HPS was bacterial and fungal infection due to immunosuppresion induced by long-term usage of the steroid, but the pathological examination suggested that cytomegalovirus infection strongly played an important role for triggering the HPS.
