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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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.
Suspect HLH In The Right Clinical Setting
- Keep HLH on your differential for adults with fever, hepatosplenomegaly, unexplained cytopenias, or elevated LFTs.
- Assess stability first by checking GCS, ABCs, vitals before proceeding with detailed workup.
HLH-2004 Criteria Are Research-Focused
- The HLH-2004 criteria require five of eight features but were designed for research and not validated in adults.
- Key criteria include fever, splenomegaly, cytopenias, hypofibrinogenemia or hypertriglyceridemia, hyperferritinemia, and elevated sIL-2R.