207. The Deadly Storm - An approach to Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH), initially recognized in children, is explored as a potentially fatal disease involving a cytokine storm. The podcast details HLH's pathogenesis, distinguishing between genetic and acquired forms, and emphasizes defective cytotoxic function as a key factor. Diagnosis relies on clinical, lab, and imaging findings, using the HLH 2004 criteria and the HScore to estimate HLH probability, while also differentiating it from conditions like Macrophage Activation System (MAS). Workup involves lab tests, bone marrow biopsies, ECGs, and imaging to identify organ involvement and occult malignancies. Treatment ranges from addressing underlying conditions to HLH-specific therapies like the HLH-94 protocol, highlighting the FDA-approved GammaFant for primary HLH and the importance of managing organ dysfunction and preventing Posterior Reversible Encephalopathy Syndrome (PRESS).