Abstract
A 6-year-old boy was admitted to our hospital for fever, anemia and hepatosplenomegaly. Laboratory data revealed pancytopenia and hyperferritinemia. A bone marrow smear demonstrated the proliferation of mature histiocytes with hemophagocytosis. The patient was diagnosed as having Epstein-Barr virus associated hemophagocytic syndrome (EBV-AHS). Massive doses of gamma-globulin were administered, but his condition worsened. He was transferred to the ICU because of disseminated intravascular coagulation syndrome and respiratory failure. Exchange transfusion and plasma exchange were performed. Dexamethasone, etoposide and cyclosporin A were administered. Respiratory and coagulative function were improved, but anemia, hepatosplenomegaly, hyperferrtinemia, increased beta2-microglobulin in the urine and mild consciousness disorder (JCS 1∼2) continued. After discharge from the ICU, transfusion intravenous dexamethasone, etoposide and cyclosporin A were necessary. Despite treatment including ACOP (adriamycin-cyclophosphamide-oncovin-prednisolone) therapy, the patient's respiration deteriorated and he died of multiple organ failure.
EBV-AHS is a rare histiocytic proliferative disorder caused by Epstein-Barr viral infection affecting children and young adults. It has a poor prognosis and there is no specific treatment. Since histiocytes can be activated by cytokines, it is said that blood purification, such as exchange transfusion and plasma exchange may reduce serum cytokines. However this case indicated that blood purification is not a reliable treatment.
