Bloody Minded Episode 32 - Phagocytose. Pray. Love. | Haemophagocytic Lymphohistiocytosis (HLH)
Jan 14, 2025
Explore the intricate world of Haemophagocytic Lymphohistiocytosis (HLH), a life-threatening condition made engaging with humor and personal stories. Discover the critical role of ferritin levels in diagnosis, key indicators like persistent fever, and the challenges of distinguishing HLH from infections. Unpack the differences between primary and secondary HLH, including genetic triggers. Learn about treatment strategies and the impact of delayed diagnosis on patient outcomes, all while keeping it light and approachable.
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HLH Pathophysiology Explained
- HLH arises when the immune system's off switch fails, causing unchecked immune activation and cytokine storms.
- This excessive response leads to harmful tissue damage and dangerous organ infiltration.
Malignancy and EBV Trigger HLH
- Malignancy, especially T-cell and NK-cell lymphomas, is a common cause of HLH in adults.
- EBV infection often triggers HLH by disrupting immune system regulation and promoting malignancy.
Use Ferritin as HLH Trigger
- Consider HLH if ferritin exceeds 10,000 mcg/L, as it's a powerful trigger signal.
- Always check ferritin early in patients with fever, pancytopenia, and organ dysfunction.