108.3 🩺 內科專科考前版

22E:
- Ropeginterferon alfa-2b (Besremi, FDA 2021) for PV — molecular response
- Pacritinib (Vonjo, FDA 2022) for MF + plt < 50K
- Momelotinib (Ojjaara, FDA 2023) for MF + anemia (also acts on hepcidin)
- MIPSS70+ v2.0 molecular prognostic for MF
- Imetelstat trials for MF (telomerase)
- Navitoclax + ruxolitinib combo trials (anti-Bcl-XL)
Taiwan: 健保 phlebotomy, aspirin, HU; 健保 anagrelide; 健保 ruxolitinib for MF + GVHD 條件; 健保 IFN-α; ropeginterferon + pacritinib + momelotinib 自費多

JAK2 V617F allele burden correlates with phenotype severity
CALR type 1 vs type 2 in ET: type 1 (52-bp deletion) more myelofibrosis-prone; type 2 (5-bp insertion) more thrombosis
Triple-negative MF: worst prognosis; consider clinical trial
Ropeginterferon (Besremi): hematologic + molecular response > HU in PROUD-PV
Ruxolitinib withdrawal syndrome: rapid splenomegaly + cytokine storm; taper
Fedratinib Wernicke encephalopathy: prophylactic thiamine 100 mg/d during therapy
Pacritinib hepcidin lower → better anemia tolerability
Momelotinib hepcidin inhibition → improves anemia (unique among JAK inhibitors)
HSCT for MF: best in DIPSS-Plus high or very high; donor availability + age + comorbidity
Splanchnic vein thrombosis (Budd-Chiari, portal) can be presenting feature of occult MPN — JAK2 V617F screening

健保 phlebotomy
健保 hydroxyurea (Hydrea)
健保 anagrelide for ET 條件
健保 IFN-α (peg-IFN) for MPN 條件
健保 aspirin
健保 ruxolitinib 條件 (MF + GVHD; PV 部分條件)
健保 HSCT for MF 條件
健保 luspatercept off-label for MF 條件
Ropeginterferon (Besremi), pacritinib (Vonjo), momelotinib (Ojjaara), fedratinib (Inrebic) 自費多 (新)
健保 JAK2 V617F + CALR + MPL panel 條件
學會: TSH (Taiwan Society of Hematology) + Taiwan MPN registry forming

⚠️ AI 草稿。