Inundate and Deluge-Hemophagocytic Lymphohistiocytosis

Abstract

Hemophagocytic lymphohistiocytosis is categorized into congenital, genetic or primary and acquired or secondary subtypes. Immune activation with consequent hyper-cytokinemia induces histiocytic infiltration within reticuloendothelial organs along with hemophagocytosis of erythrocytes, leukocytes, platelets and precursor cells. Untreated primary and secondary hemophagocytic lymphohistiocytosis is invariably associated with disease associated mortality. However, adoption of contemporary therapeutic protocols document 5 year survival rates of ~ 54%. Familial or primary hemophagocytic lymphohistiocytosis is a progressive, autosomal recessive condition characteristically occurring within young age. The disorder exemplifies decimated NK cell function and frequently detected mutations within perforin gene.