Overview
Definition
Myeloproliferative neoplasm characterized by overproduction of RBCs.
Epidemiology
Peaks in 60s, slightly more common in men.
Etiology & Risk Factors
- JAK2 V617F mutation (in >95% of cases)
Clinical Symptoms
- Pruritus after hot shower (aquagenic pruritus)
- Plethora (red face)
- Headache
- Erythromelalgia (burning pain in hands/feet)
Clinical Approach
Diagnosis
- High Hemoglobin/Hematocrit
- Low Erythropoietin (EPO)
- Positive JAK2 mutation
Management
- Phlebotomy (keep Hct < 45%)
- Hydroxyurea
- Low-dose aspirin
Complications
- Arterial/Venous thrombosis
- Progression to myelofibrosis
- Transformation to Acute Myeloid Leukemia (AML)