Hemophagocytosis in the Acute Phase of Fatal Kawasaki Disease in a 4 Month-Old Girl

Abstract

Background: Kawasaki disease is a systemic vascu-litis predominately affecting coronary arteries. Hemo-phagocytic lymphohistiocytosis can complicate the course of Kawasaki disease. Rare cases of secondary hemophagocytic lymphohistiocytosis occurring dur-ing the acute phase of Kawasaki disease have been reported.Case Report: We report here a 4 month-old girl with diffuse coronary ectasia and secondary hemophago-cytic lymphohistiocytosis occurring during the acute phase of incomplete Kawasaki disease.Conclusion: Due to the large overlap in clinical symp-toms, the presence of atypical findings for Kawasaki disease should suggest the possible diagnosis of he-mophagocytic lymphohistiocytosis in these patients.Keywords: Coronary artery dilatation, hemophago-cytic lymphohistiocytosis, Kawasaki disease, macro-phage activation syndrome