ð åèçïŒé«åž«åè / PGY OSCEïŒ
ð Cram Sheet
ð¥ é« yield 8
- HLH-2004 criteria 5/8
- Ferritin ⥠500 clinical / > 10,000 highly specific
- Primary å
ç«¥ genetic (PRF1, UNC13D, STX11)
- Secondary æäºº â infection / cancer / autoimmune (MAS)
- HLH-94: etoposide + dex + IT MTX
- MAS = Stillâs, SLE, Kawasaki
- Ruxolitinib emerging
- Anakinra for MAS + rituximab for EBV-HLH
ð¢ å¿
è
| Ferritin clinical cutoff |
⥠500 |
| Ferritin high specific |
> 10,000 |
| Cytopenia |
⥠2 lineage |
| Hgb cutoff |
< 9 |
| Plt cutoff |
< 100 |
| ANC cutoff |
< 1 |
â é« yield
HLH-2004 (5/8)
- Fever
- Splenomegaly
- Cytopenia ⥠2 lineage
- Hypertriglyceridemia / hypofibrinogenemia
- Ferritin ⥠500
- Hemophagocytosis BM
- Low NK
- High sCD25
Adult Secondary HLH Triggers
- Infection: EBV (most), CMV, dengue, malaria, parvovirus, HIV, COVID
- Cancer: T-cell lymphoma (most), B-NHL, AML
- Autoimmune (MAS): Adult-onset Stillâs, SLE, RA
- Drug-induced (rare)
MAS Specific
- åš autoimmune setting
- Often Stillâs disease (juvenile or adult-onset)
- Treat: high-dose steroid + cyclosporine + anakinra (IL-1 antagonist)
EBV-Driven HLH
- High EBV viral load
- Rituximab + HLH-94
ð¯ èªææª¢æž¬
- HLH-2004 criteria æž? â 5/8
- Ferritin highly specific? â > 10,000
- Primary HLH genes 3? â PRF1, UNC13D, STX11
- Secondary äžé¡ trigger? â Infection / cancer / autoimmune
- HLH-94 äžè¥? â Etoposide + dex + IT MTX
- MAS first-line additional? â Anakinra
- EBV HLH å ? â Rituximab
- Ruxolitinib target? â JAK1/2 (22E emerging)
â ïž AI èçš¿ã