Abstract
We examined changes in the serum cytokine concentration in a patient with refractory Epstein-Barr virus-associated hemophagocytic syndrome. A 30-year-old man was admitted to a hospital with severe infection and disseminated intravascular coagulation. Since his condition progressed to severe acute renal failure, he was transferred to our hospital. He underwent continuous hemodiafiltration for the renal failure, under artificial respiration. He was suspected of having hemophagocytic syndrome (HPS) on the basis of abnormal clinical symptoms and laboratory data obtained on the following day, and diagnosed as having HPS from the result of a bone marrow aspiration. We began steroid pulse therapy and plasma exchange (PE) but did not perform immunochemotherapy because of severe liver dysfunction. High concentration of serum cytokines at the onset of treatment suggested an adverse prognosis. The cytokine balance indicated immunoparalysis with predominance of anti-inflammatory cytokines. Although the cytokine balance was improved after steroid pulse therapy and PE, the clinical symptoms were not improved. The present case suggested that early treatment and effective cytokine modulation were necessary for severe cases of HPS.
