The Intern At Work: Internal Medicine 207. The Deadly Storm - An approach to Hemophagocytic Lymphohistiocytosis
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Sep 17, 2023 Explore the complexities of Hemophagocytic Lymphohistiocytosis (HLH), a serious immune disorder. Discover the differences between primary and secondary HLH and the latest methods for diagnosis and management. The discussion includes insights into recent clinical trials that shed light on treatment advancements. This deep dive reveals the condition's pathophysiology, illustrating how it manifests in both children and adults.
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HLH Is Not Just A Pediatric Disease
- HLH was first described as a childhood immune dysregulatory disorder but can present at any age.
- Both familial and sporadic forms cause uncontrolled lymphocyte and macrophage activation leading to a cytokine storm.
Perforin Failure Drives Genetic HLH
- Genetic HLH results from defective cytotoxic function, often due to perforin mutations.
- Failure to kill antigen-presenting cells causes persistent immune stimulation and failure of immune contraction.
Multiple Routes Lead To The Same Storm
- Acquired (secondary) HLH arises from diverse mechanisms including immunosuppression, rheumatologic triggers, and SNPs.
- Different pathways converge on the same hyperinflammatory phenotype seen in primary HLH.