Hemophagocytic Lymphohistiocytosis: A Case Series

Hemophagocytic lymphohistiocytosis (HLH) has been recognized as an inflammatory endpoint for a variety of conditions including autoimmune diseases, malignancies and infections. It can be further classified as primary and secondary HLH. Primary HLH is also known as familial HLH. It usually presents in childhood and can be associated with gene mutations. Secondary HLH is also known as acquired HLH and usually presents in adulthood. In comparison to children, it is difficult to diagnose adults with HLH since it occurs with a variety of different diseases and most of the literature on HLH is derived from a pediatric population. In this case series, we report two cases of HLH that illustrate the difficulty of making this diagnosis and a brief review of the literature on its pathophysiology, clinical presentation, diagnosis, and a subsequent therapeutic approach.