[A case of IAHS (infection associated hemophagocytic syndrome) successfully treated with etoposide]

This report details a case of infection associated hemophagocytic syndrome (IAHS). A 20-year-old female was admitted to our hospital with persistent high fever in July, 1994. Physical examination revealed high body temperature (40 degrees C), marked hepatosplenomegaly and no superficial lymph node swelling. Laboratory examination revealed leukopenia and abnormal liver function on admission. Serum ferritin levels were surprisingly elevated. The coagulation tests showed high FDP and D-dimer. Specific viral antibody titers were not elevated such as Epstein-Barr virus or Cytomegalovirus. Bone marrow examination revealed histiocytic hyperplasia with hemophagocytosis, and the histiocytes were well matured. We diagnosed IAHS. Corticosteroids were administered on the 3rd hospital day (methylprednisolone 1 g/day, 3 days), but persistent high fever and laboratory findings did not improve. So we tried etoposide (etoposide 200 mg/day, 5 days) therapy on the 13th hospital day. After administration of etoposide, she failed to recover from severe leukopenia and suffered from meningitis. We administered G-CSF, gamma-globulin and antibiotics for intensive supportive therapy. As the leukocyte count increased, her symptoms and laboratory data improved. There was no hemophagocytosis in her bone marrow before discharge. Recently, etoposide is said to be effective for reactive monocytic proliferation. Administration of etoposide was very effective for IAHS, although corticosteroids, were ineffective.