A 6-year-old boy developed hemophagocytic syndrome during the recurrent cou
rse of Kawasaki disease. Despite the appropriate treatment modalities for K
awasaki disease, he developed pancytopenia, marked hepatosplenomegaly, high
-grade fever, hyperferritinemia, hypertriglyceremia, and evidence of hemoph
agocytosis in the liver biopsy. Although the course was stormy, he responde
d well to a combination therapy of corticosteroids, etoposide VP16, and gra
nulocyte colony-stimulating factor G-CSF. The clinical course and the treat
ment given were compared with the previous reported cases.