68.3 🩺 內科專科考前版

68.3.0.1 📌 䞀頁重點

  • 22E: ruxolitinib for adult HLH, emapalumab (anti-IFN-γ) for primary HLH refractory
  • Adult HLH-2004 + H-Score
  • Taiwan: EBV / dengue / autoimmune triggers

68.3.0.2 🌟 Pearls (8)

  1. Adult HLH H-Score > 169 high probability
  2. Emapalumab (anti-IFN-γ) for primary HLH refractory
  3. Ruxolitinib small RCTs in adult HLH promising
  4. Anakinra for MAS standard
  5. Rituximab + HLH-94 for EBV-driven
  6. HSCT for refractory primary HLH definitive
  7. CAR-T cytokine release syndrome related to HLH
  8. COVID-19 cytokine storm HLH-like → tocilizumab evidence

68.3.0.3 📍 Taiwan

  • EBV high → EBV-HLH 譊芺
  • Dengue endemic → dengue HLH
  • Adult-onset Still’s → MAS
  • 健保 IVIG / steroid / etoposide
  • 健保 anakinra / ruxolitinib 限制

68.3.0.4 🎓 內專重點

  1. HLH-2004 + H-Score
  2. Primary vs secondary
  3. MAS treatment
  4. Trigger-specific treatment
  5. New drugs (ruxolitinib, emapalumab)

⚠ AI 草皿。