Hemophagocytic Lymphohistiocytosis: an update to diagnosis and management

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is the uncontrolled reaction of the immune system against a triggering pathogen and inability of the im- mune system to elliminate this triggering factor, which ends up with hyper- cytokinemia and hemophagocytosis. Hemophagocytic lymphohistiocytosis is classified into two major groups as genetic (primary) and acquired (sec- ondary). The condition has high mortality rates and specific tratment is re- quired in most of the cases