Virus associated hemophagocytic syndrome (VAHS) is a disorder characte
rized by a benign, generalized histiocytic proliferation with bone mar
row and liver disorder and marked hemophagocytosis associated with sis
temic infections; VAHS is a rare illness, probably understimated, ther
efore we think it convenient to describe this case with rapidly fatal
outcome, recently observed. A 2-year-old girl was admitted to our hosp
ital with 2 weeks of persistent fever, hepatosplenomegaly, jaundice, s
opor; laboratory studies revealed an acute Epstein-Barr virus infectio
n and a bone marrow aspirate with proliferation of histiocytes showing
hemophagocytosis; the outcome was quick and it was possible just the
start of chemotherapy.