A Case of Bacteria-associated Hemophagocytic Syndrome Complicated by Multiple Organ Dysfunction. That was Successfully Treated Using Plasma Exchange.

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A 55-year-old woman was admitted to our hospital for somnolence and fever (38°C). A chest computed tomography (CT) scan performed at the time of admission showed pneumonia. A blood test showed pancytopenia. The patient received artificial respiration in the ICU. Steroid and antibiotic medication were administrated. However, the patient's blood findings deteriorated. The serum ferritin level was markedly elevated to 14, 379ng/ml. A bone marrow smear showed 4.6% hemophagocytosis. A bacterial culture of the sputum was positive for Pseudomonas aeruginosa. Under a diagnosis of bacteria-associated hemophagocytic syndrome (HPS), plasma exchange (PE) was performed. After PE, the hematological data improved markedly. The patient was extubated on the 5th hospital day and discharged on the 27th hospital day. To diagnosis HPS, hyperferritinemia and evidence of hemophagocytosis from a bone marrow biopsy should be confirmed. However, the etiology of HPS can vary, and a standard treatment has not been established. PE has been reported to be useful for removing cytokines. However, the mechanism behind the efficacy of PE remains to be clarified. In patients with HPS, treatment using PE, steroids and immunosuppressive agents should be started as early as possible, preferably before the development of hypercytokinemia-related multiple organ failure.
一般社団法人日本救急医学会の論文