腹部症状で発症し急激な経過をたどり死亡した血球貪食症候群の1例

抄録

A 52-year-old woman with abdominal symptoms associated with severe ascites and splenomegaly developed dyspnea and consciousness disorder and was transferred from another hospital. On admission, her platelet count was very low, her clotting function had failed, and she had developed disseminated intravascular coagulation (DIC). We treated DIC symptomatically while searching for the etiology. When the patient's renal failure, dyspnea, and bleeding diathesis rapidly worsened, she developed multiple organ failure. Circulatory maintenance by continuous hemodiafiltration succeeded only temporary and she died on hospital day 5. Autopsy showed that hemophagocytic histiocytes had infiltrated organs and blood vessels throughout the body, and the final diagnosis was hemophagocytic syndrome secondary to Epstein-Barr virus associated with peripheral T-cell lymphoma (lymphoma-associated HPS: LAHS), based on immunostaining results. Severe cases of LAHS pursue a fulminant course, often leading to death. HPS must be kept in mind in the differential diagnosis of treatmentresistant cases giving rise to fever of unknown origin, pancytopenia, and splenomegaly. Determining the cause of HPS and conducting adequate chemotherapy immediately after diagnosis are vital to saving the patient's life.