Hemophagocytic lymphohistiocytosis: pouring gasoline on the cytokine storm

Abstract

Hemophagocytic lymphohistiocytosis should be considered in patients with persistent fever, hepatosplenomegaly, pancytopenia. Hypercytokinemia originated from genetic disorder effecting the cellular defects of cytotoxic T and natural killer lymphocyte activity is the underlying pathophysiology of the disorder. In this review we summarized the recent advances in the management of hemophagocytic lymphohistiocytosis.