Two separate episodes of hemophagocytic syndrome at a two-year interval inan apparently immunocompetent male

We describe two separate episodes of hemophagocytic syndrome (HPS) at an in terval of two years in a seemingly immunocompetent male. This case suggests the possible existence of an inherent predisposition to HPS, in which othe rwise negligible self-limited viral infection may trigger HPS. Laboratory d ata for a 16-year-old boy admitted with persistent high grade fever and sev ere thrombocytopenia disclosed coagulation abnormality, liver damage, and h ypercytokinemia. A bone marrow aspiration revealed a proliferation of histi ocytes with fresh hemophagocytosis. We diagnosed that he was suffering from HPS. Responding to steroid pulse therapy, he recovered completely and was discharged. After two years of healthy life, he became febrile again and wa s readmitted. The fever was refractory to antibiotics and was associated wi th a sudden drop in platelet count. Laboratory data and the bone marrow pic ture were consistent with those of HPS. He was again successfully treated w ith steroid. After the second episode, he has been healthy for more than tw o years. (C) 1999 The Japanese Society of Hematology.