Presentation :
- Many patients have symptoms of fatigue, headache, ruddy complexion,
- Itching, particularly after a warm shower (aquagenic pruritus).
- Accompanying splenomegaly may cause abdominal fullness, reflux, or early satiety; some patients may have a ruddy complexion.
- Splanchnic vein thrombosis, and portal or hepatic vein thrombosis may be the presenting feature (sometimes preceding hematologic manifestations).
- This is very rare, and associated with only a few other conditions. #todo
Pathophysiology :
Diagnostics :
- Erythrocytosis (>16.5 g/dL in men, >16 g/dL in women)
- Thrombocytosis and neutrophilia are commonly seen as well
- Must exclude secondary causes of erythrocytosis (more common than p vera)
- Chronic hypoxia (smoking, OSA)
- Tests: Sleep study, pulse ox
- Ectopic erythrocyte production (most commonly Renal cell carcinoma (RCC))
- Exogenous testosterone usage
- JAK2 mutation positivity
Treatment :
- Phlebotomy to maintain a hematocrit level less than 45%
- Low-dose aspirin to reduce thrombotic risk
- In pts at higher risk (age >60, hx MI/stroke/VTE) can use cytoreductive agents like Hydroxyurea and IFN-alpha
- The JAK1/2 inhibitor ruxolitinib is used in patients with resistant PV or who cannot tolerate first-line agents
- Patients with venous thromboembolism will generally require life-long anticoagulation.
Prognosis: