69.3 🩺 內科專科考前版

69.3.0.1 📌 䞀頁重點

  • 22E: emicizumab + gene therapy hemophilia, andexanet alfa for Xa, caplacizumab for TTP, fostamatinib for ITP
  • DOAC dominance + 高 reversal awareness
  • Taiwan: 健保 DOAC 普及

69.3.0.2 💊 進階

69.3.0.2.1 Hemophilia New Therapies
  • Emicizumab (anti-FIXa/FX bispecific Ab) — q1-4 wk SC, prophylaxis revolution
  • Gene therapy:
    • Roctavian (valoctocogene roxaparvovec) AAV5 — hemophilia A
    • Hemgenix (etranacogene dezaparvovec) AAV5 — hemophilia B
  • Concizumab (anti-TFPI Ab) emerging
  • Fitusiran (siRNA antithrombin) emerging
69.3.0.2.2 Hemophilia with Inhibitor
  • Bypassing agents: rFVIIa (NovoSeven), aPCC (FEIBA)
  • Emicizumab highly effective in inhibitor patients
69.3.0.2.3 ITP New Drugs
  • Fostamatinib (SYK inhibitor) — refractory
  • Avatrombopag TPO-RA
  • Rilzabrutinib (BTK inhibitor) emerging
69.3.0.2.4 TTP
  • Caplacizumab (anti-vWF nanobody) + PLEX + steroid
  • ADAMTS13 monitoring
  • HSCT in genetic forms
69.3.0.2.5 APS
  • Warfarin INR 2-3 (or 3-3.5 for arterial)
  • DOACs NOT preferred (TRAPS trial — rivaroxaban worse)
  • Hydroxychloroquine adjunct
  • Pregnancy: aspirin + LMWH
69.3.0.2.6 DOAC Caveats
  • Avoid in: mechanical valve, antiphospholipid syndrome, pregnancy, severe renal impairment (varies)
  • Renal adjustment crucial
69.3.0.2.7 HIT
  • 4T → ELISA → SRA (gold standard)
  • Argatroban (preferred renal failure), bivalirudin (cardiac surgery), fondaparinux (off-label)
  • Avoid LMWH cross-reactivity

69.3.0.3 🌟 Pearls (10)

  1. Emicizumab prophylaxis transforms hemophilia A care
  2. Roctavian / Hemgenix gene therapy 2022/2023 approved
  3. Caplacizumab + PLEX + steroid is standard TTP
  4. Fostamatinib for refractory ITP
  5. DOAC TRAPS trial: NOT for APS
  6. Warfarin in mechanical valve still standard
  7. Bridging anticoagulation declining (BRIDGE trial)
  8. ECT (ecarin clotting time) for dabigatran specific
  9. Trousseau syndrome = malignancy + migratory phlebitis → LMWH > warfarin
  10. APL DIC + ATRA within hours of suspicion (don’t wait for confirm)

69.3.0.4 🔍 特殊情境

69.3.0.4.1 Pregnancy Anticoagulation
  • LMWH first-line (no placental crossing)
  • Warfarin teratogenic 1st trimester + bleeding 3rd
  • DOAC 䞍掚薊
69.3.0.4.2 Cancer-Associated VTE
  • LMWH classic
  • DOACs (apixaban, edoxaban, rivaroxaban) increasing evidence
  • Caution GI cancer (rivaroxaban GI bleed)
69.3.0.4.3 ICU Anticoagulation
  • VTE prophylaxis (LMWH or UFH)
  • ECMO heparin
  • COVID-19 anticoagulation guidelines

69.3.0.5 📍 Taiwan

  • 健保 DOACapixaban, rivaroxaban, dabigatran, edoxabanfor AF/VTE
  • 健保 emicizumab for hemophilia A
  • Gene therapy 自費 (極高)
  • 健保 caplacizumab for TTP

69.3.0.6 🎓 內專重點

  1. PT/aPTT 鑑別
  2. Hemophilia 新藥 (emicizumab, gene therapy)
  3. vWD types
  4. DIC + APL emergency
  5. APS criteria + warfarin
  6. HIT 4T + argatroban
  7. DOAC reverse
  8. ITP refractory new drugs

⚠ AI 草皿。