IBCC Episode 104 - Hemophagocytic LymphoHistiocytosis (HLH)

In this episode of The Internet Book of Critical Care Podcast, Josh and Adam delve into hemophagocytic lymphohistiocytosis (HLH), describing it as an immune dysregulation involving CD8 positive T cells, macrophages, and cytokine release, which can be primary (genetic) or secondary (triggered by infection or other factors). They discuss clinical features such as persistent fever, CNS dysfunction, shock, multi-organ failure, and organomegaly, as well as lab findings like cytopenias, abnormal liver enzymes, hypertriglyceridemia, and coagulation abnormalities. They also cover diagnostic challenges, including the limitations of hemophagocytosis as a marker, and address potential causes such as rheumatologic disorders, malignancies, infections, and immune compromise. The discussion further explores treatment strategies, emphasizing the importance of addressing the underlying trigger, the use of steroids, IL-1 antagonists like Anakinra, JAK inhibitors, and chemotherapeutic agents like etoposide, while highlighting the need for a multi-disciplinary approach and caution in immunosuppressive therapies.