Pb2045: Use of Anakinra in the Treatment of Adult Hemophagocytic Lymphohistiocytosis: A Systematic Review

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome characterized by extreme inflammation and tissue destruction through immune activation of T cells, NK cells, and macrophages leading to a cytokine storm. HLH is traditionally classified as primary and secondary. While primary HLH is typically familial and seen in pediatric populations, secondary HLH is often caused by autoimmune diseases, infectious diseases, and malignancies. While underlying incidence is very low, HLH is associated with significant mortality reaching near 100% when untreated, and 50-75% with traditional chemotherapy-based treatment regimens. Recently, treatment regimens have increasingly included Anakinra, an IL-1 Receptor antagonist. The evidence for the use of Anakinra is primarily founded upon pediatric literature, while there is very limited evidence for its use in adult patients.