Hemophagocytic syndrome with restricted organ involvement: excessive hemosiderosis and fibrosis of the spleen

Abstract

We report the case history of a 6 1/2-month-old girl with a hemophagocytic syndrome, pancytopenia, and excessive hepatosplenomegaly. Some extraordinary histological features present in this case--restricted organ involvement, excessive hemosiderosis, and fibrosis of the spleen--further contributed to the well-known problem of distinguishing between infection-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis.