107.3 🩺 內科專科考前版

107.3.0.1 📌 䞀頁重點

  • 22E:
    • Eltrombopag added to ATG + cyclosporine in AA: better response (Townsley NEJM 2017; updated 2022)
    • Luspatercept (Reblozyl) expanded 2023 for all lower-risk MDS (not just SF3B1)
    • Imetelstat (Rytelo, FDA 2024) telomerase inhibitor for transfusion-dependent lower-risk MDS refractory to ESA
    • Venetoclax + AZA for MDS (similar to AML)
    • IPSS-M (molecular) 2022 prognostication
    • WHO 2022 classification major update
  • Taiwan: 健保 ATG, cyclosporine, eltrombopag, lenalidomide, azacitidine 條件; HSCT 條件; luspatercept (新, 條件); imetelstat 自費 倚

107.3.0.2 🌟 Pearls (10)

  1. Horse ATG > rabbit ATG for SAA (NEJM 2011)
  2. Eltrombopag (Promacta) TPO-R agonist; added to ATG/CSA improves response (~ 90% from 60%)
  3. PNH clone in AA: 50%+ AA have small PNH clone (FLAER+); doesn’t change initial AA management
  4. MDS-RS + SF3B1: better prognosis than non-mutant
  5. TP53 in MDS: subdivided into mono-allelic (better) vs bi-allelic (worse, AML-like)
  6. CHIP (clonal hematopoiesis of indeterminate potential) vs MDS: CHIP < 2% blasts + no cytopenia + no dysplasia
  7. Azacitidine 7-day vs 5-day: standard 7 days; 5-day off-label sometimes
  8. Decitabine 5-day vs 10-day: 10-day for TP53-mutant (DAUNTLESS trial limited)
  9. Venetoclax + AZA in MDS: trials ongoing; AML data positive (VIALE-A)
  10. Imetelstat telomerase inhibitor: novel mechanism; for 5q− negative ESA-refractory

107.3.0.3 📍 Taiwan + 健保

  • 健保 ATG (horse) + cyclosporine + eltrombopag for AA 條件
  • 健保 HSCT (matched sibling + MUD) 條件
  • 健保 lenalidomide for del(5q) MDS 條件
  • 健保 luspatercept (Reblozyl) for MDS 條件 (新)
  • 健保 azacitidine + decitabine 條件
  • 健保 venetoclax + AZA combo 條件 (AML; MDS off-label)
  • Imetelstat (Rytelo) 自費 倚 (新, 條件 limited)
  • 健保 IPSS-R + 郚分 IPSS-M molecular panel 條件
  • 孞會: TSH (Taiwan Society of Hematology); MDS Foundation

107.3.0.4 🎓 內專必懂 (10)

  1. AA Camitta + IST + eltrombopag (NEJM 2017+)
  2. HSCT vs IST decision (age, donor, severity)
  3. MDS WHO 2022 + IPSS-M molecular prognostication
  4. del(5q) lenalidomide + TP53 caveat
  5. Lower-risk MDS treatment ladder (ESA → luspatercept → imetelstat 22E)
  6. Higher-risk MDS (AZA/decitabine + venetoclax + HSCT)
  7. Hereditary BMF syndromes recognition
  8. Fanconi DEB test
  9. PNH clone in AA + flow cytometry
  10. CHIP vs MDS distinction

⚠ AI 草皿。