Successful treatment of infection-associated hemophagocytic syndrome with intravenous immunoglobulin

Hemophagocytic syndrome is a rare disorder characterized by a group of clinical, laboratory and histopathological findings such as fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, and hemophagocytosis in the bone marrow, spleen, and lymph nodes. Hemophagocytic syndrome may occur as a primary or secondary disease. Primary type of hemophagocytic syndrome is also known as familial erythrophagocytic lymphohistiocytosis and secondary type is mostly associated with an viral infection and known as infection-associated hemophagocytic syndrome (IAHS). Rapid diagnosis is very important in these patients since suggested treatment strategies for the two types have been different and mortality rate is very high. In this report we present the clinical and laboratory findings and the outcome of two children with IAHS to emphasize the importance of early diagnosis and the effectiveness of intravenous immunoglobulin therapy in these patients