ABSTRACT

Hemophagocytic lymphohistiocytosis is a rare, life-threatening disorder characterized with uncontrolled activation of T-helper 1 lymphocytes and macrophages and over-release of inflammatory cytokines. In this paper, we presented two patients who admitted to our hospital due to abdomen swelling, pallidness, febrility, hepatosplenomegaly and pancytopenia and diagnosed as familial hemophagocytic lymphohistiocytosis.