Nathan brought in a great case of a 38yo male with recently diagnosed autoimmune hepatitis who presented with worsening abdominal swelling, found to have Budd-Chiari Syndrome due to polycythemia vera!
Learning Points:
Other learning points:
- Causes of Budd-Chiari Syndrome
- Primary
- Malignancy
- Myeloproliferative disease (PV, CML, etc)
- PNH
- APS
- Protein C, S deficiency
- Antithrombin III
- Secondary
- Extrinsic compression or infiltration
- Treatment of Polycythemia Vera
- Low risk: Age <60 without thrombosis
- Aspirin, phlebotomy (goal HCT<45)
- High risk: Age >60 with/without thrombosis
- Aspirin, phlebotomy, hydroxyurea
- Treatment of Budd-Chiari thrombosis
- Anticoagulation (warfarin vs LMWH)
- Thrombectomy/thrombolysis
- Treat underlying cirrhosis/transplant
- TIPS
- Low risk: Age <60 without thrombosis
- Primary