The hemophagocytic syndrome/macrophage activation syndrome: a final common pathway of a cytokine storm.

摘要

The hemophagocytic syndrome, or hemophagocytic lymphohistiocy-tosis, is a clinical syndrome associated with a variety of underlying conditions leading to a severe inflammatory response. This inflammatory response is induced by a "cytokine storm" and is characterized by the proliferation and activation of macrophages in the reticuloendothelial system, causing lymphadenopathy, hepatosplenom-egaly and pancytopenia. The hallmark of this syndrome is the impairment or absence of natural killer and cytotoxic T cell function. The uncontrolled and ineffective immune activation results in multiorgan dysfunction.