Hemophagocytic Lymphohistiocytosis: an update to diagnosis and management

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