66.3 🩺 內科專科考前版

66.3.0.1 📌 䞀頁重點

  • 22E: caplacizumab for TTP, voxelotor/crizanlizumab/L-glutamine for SCD, ruxolitinib for PV/MF
  • HIF-PHI (roxadustat) for CKD anemia (China/Asia approved)
  • Taiwan: thalassemia 高 prevalence

66.3.0.2 💊 進階

66.3.0.2.1 TTP Modern Management
  • PLEX (plasma exchange) + steroid + caplacizumab (anti-vWF nanobody)
  • Rituximab for refractory / relapsed
  • ADAMTS13 monitoring
66.3.0.2.2 Sickle Cell New Drugs
  • Hydroxyurea classic
  • Voxelotor (HbS polymerization inhibitor)
  • Crizanlizumab (anti-P-selectin Ab)
  • L-glutamine
  • Gene therapy (Casgevy CRISPR, Lyfgenia lentiviral)
66.3.0.2.3 CKD Anemia
  • ESA (epoetin, darbepoetin)
  • IV iron
  • HIF-PHI: roxadustat, daprodustat, vadadustat (oral)
66.3.0.2.4 Aplastic Anemia
  • Triple immunosuppression: ATG + cyclosporine + eltrombopag (TPO-receptor agonist)
  • HSCT for young + severe + matched donor
66.3.0.2.5 Polycythemia Vera
  • Phlebotomy (Hct < 45%)
  • Aspirin
  • Hydroxyurea
  • Ropeginterferon-α2b (Besremi) — 22E new
  • Ruxolitinib for HU-resistant
66.3.0.2.6 Pure Red Cell Aplasia
  • Parvovirus B19 (in immunocomp)
  • Thymoma
  • Drugs

66.3.0.3 🌟 Pearls (10)

  1. TTP caplacizumab + PLEX + steroid (HERCULES trial)
  2. PNH FLAER + CD55/59 flow cytometry diagnostic
  3. MDS diagnostic: cytogenetics + IPSS-R + WHO
  4. HU > anti-IL-6 for symptomatic PV pruritus
  5. Sickle cell hyperbaric / exchange transfusion for stroke prevention
  6. HIF-PHI: oral CKD anemia, but cardiovascular monitoring
  7. Eltrombopag for aplastic anemia + ITP
  8. Aplastic horse-ATG > rabbit-ATG (overall survival)
  9. CALR + MPL mutations in MPN (PV/ET/MF)
  10. Voxelotor in SCD: Hgb ↑ but VOC reduction limited

66.3.0.4 📍 Taiwan

  • Thalassemia carrier screening 普及
  • G6PD newborn screening
  • 健保 hydroxyurea for SCD/PV
  • 健保 ESA for CKD
  • DAA HCV → reduced 郚分 hepatic anemia

66.3.0.5 🎓 內專重點

  1. MCV / retic 分類
  2. IDA workup + Tx (22E alternate day)
  3. B12/folate
  4. Hemolytic anemia 分類
  5. TTP / TMA syndromes
  6. PV / aplastic
  7. SCD new drugs (22E)

⚠ AI 草皿。