108.2 📚 國考版醫垫國考 / PGY OSCE

108.2.0.1 📌 Cram Sheet

108.2.0.1.1 🔥 高 yield 12
  1. MPN 3 倧: PV / ET / MF
  2. JAK2 V617F: 95% PV / 50-60% ET / 50% MF
  3. CALR: 20-30% ET / 25% MF (better prognosis)
  4. MPL: 3-5% ET / 8% MF
  5. PV diagnosis: Hb > 16.5/16 + JAK2 + low EPO
  6. PV high-risk: > 60 OR prior thrombosis
  7. PV Tx: phlebotomy (Hct < 45/42) + aspirin + HU/IFN/ruxolitinib
  8. Ropeginterferon alfa-2b (Besremi): PV (FDA 2021)
  9. ET high-risk Tx: aspirin + HU; anagrelide 2nd
  10. MF treatment: ruxolitinib + HSCT (curative)
  11. Fedratinib + B1 (Wernicke watch)
  12. Pacritinib (plt < 50K) + momelotinib (anemia) 22E
108.2.0.1.2 🔢 必背
項目 æ•žå­—
PV Hb 男/女 > 16.5 / 16
PV Hct 男/女 > 49% / 48%
Phlebotomy target Hct < 45 男 / < 42 女
ET Plt > 450K
Very high plt + aspirin caution > 1500K

108.2.0.2 ⭐ 高 yield

108.2.0.2.1 Mutation Quick
Disease JAK2 V617F JAK2 exon 12 CALR MPL Triple neg
PV 95% 3% < 1% < 1% rare
ET 50-60% < 1% 20-30% 3-5% 10%
MF 50% rare 25% 8% 15%
108.2.0.2.2 JAK Inhibitors
Drug Target Use
Ruxolitinib JAK1/2 MF + PV (HU-R) + GVHD
Fedratinib JAK2 MF 2nd; B1 watch
Pacritinib JAK2/IRAK1 MF + plt < 50K
Momelotinib JAK1/2 + ACVR1 MF + anemia
Tofacitinib JAK1/3 RA, IBD (not MPN)
108.2.0.2.3 Other Drugs
  • Hydroxyurea (Hydrea): 1st cytoreduction
  • Anagrelide: PDE3 inhibitor; ET ↓ plt; cardiac caveat
  • IFN-α / Pegylated IFN / Ropeginterferon alfa-2b (Besremi): molecular response; pregnancy
  • Aspirin: 81 mg/d
  • Luspatercept: MF anemia (off-label)
  • Danazol: MF anemia (older)
108.2.0.2.4 Risk Scores
  • PV: ELN risk (age + thrombosis history)
  • ET: IPSET-thrombosis (age, JAK2, CV risk, prior thrombosis)
  • MF: DIPSS-Plus, MIPSS70+
108.2.0.2.5 Transformation Risk
  • PV → MF: 10% / 15 yr
  • PV → AML: 5-10%
  • ET → MF: 5-10%
  • ET → AML: < 5%
  • MF → AML: 20-30% blast-phase (worst)

108.2.0.3 🎯 自我檢枬

  1. PV mutation #1? → JAK2 V617F (95%)
  2. ET mutations 3? → JAK2, CALR, MPL
  3. CALR prognosis? → Better
  4. PV phlebotomy target? → Hct < 45 男 / < 42 女
  5. PV high-risk Tx? → Phlebotomy + aspirin + HU
  6. Ropeginterferon alfa-2b? → PV (Besremi, FDA 2021)
  7. ET high plt aspirin caveat? → > 1500K (vWF def → bleeding)
  8. MF curative? → HSCT
  9. MF symptom control? → Ruxolitinib
  10. Fedratinib SE? → Wernicke (B1 supplementation)
  11. Pacritinib indication? → MF + plt < 50K
  12. Momelotinib indication? → MF + anemia
  13. MF smear classic? → Dacrocytes (teardrop) + leukoerythroblastic
  14. MF BM? → Fibrosis + atypical megakaryocyte
  15. PV pruritus characteristic? → Aquagenic (post-bath)

⚠ AI 草皿。