真菌性敗血症に合併した血球貪食症候群の一例

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  • A case of hemophagocytic syndrome associated with fungal sepsis

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A case of hemophagocytic syndrome (HPS) associated with fungal sepsis is described. A 64-year-old male was admitted to our hospital because of acute abdomen. He was diagnosed as having intestinal perforation and underwent an urgent intra-abdominal operation that involved irrigation, drainage and omentopexy. During and after the operation, hypotension persisted despite vigorous fluid therapy and administration of a vasopressor. On ICU admission, oligouria persisted and continuous hemodiafiltration (CHDF) was therefore started. Empiric use of antibiotics was also started for the treatment of sepsis syndrome. On the 3rd ICU day, the culture of ascites in the operation was positive for Candida albicans, and an antifungal agent was administered. After ICU admission, thrombocytopenia and anemia, which required supplements of concentrated platelet and packed red cells, and liver dysfunction gradually developed. On the 9th ICU day, platelet count had decreased to 17, 000·μl-1, serum ferritin level had increased to 3, 000μg·ml-1 and total bilirubin level had increased to 34mg·ml-1. Examination of bone marrow obtained by aspiration performed on the 9th ICU day showed significant hemophagocytosis. Steroid pulse therapy was then started, and hematological data associated with HPS improved in response to the steroid therapy. However, the patient died on the 30th postoperative day. It was thought that in addition to the steroid therapy, CHDF for renal impairment played a role in the temporary improvement of the symptoms of HPS associated with fungal sepsis. When pancytopenia is seen in a patient with sepsis, a differential diagnosis of HPS should be made immediately.

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