Hemophagocytic Lymphohistiocytosis Case Series

Hemophagocytic Lymphohistiocytosis (HLH) is a rare, lifethreatening syndrome of excessive multisystem inflammation and tissue destruction due to abnormal immune activation. It is more frequently seen in the pediatric population, either as a genetic disorder or secondary to infection or autoimmune disease, but can present in acquired forms at any age. It is thought to be due to an absence of downregulation of activated macrophages by Natural Killer (NK) cells and/or cytotoxic lymphocytes. This then leads to excessive macrophage activity and results in cytokine storm and tissue damage [1-3].