hematology and oncology
Polycythemia Vera
Symptoms and Signs of Polycythemia Vera
Polycythemia vera itself is often asymptomatic, but eventually the increased red cell volume and viscosity cause weakness, headache, light-headedness, visual disturbances, fatigue, and dyspnea. Pruritus often occurs, particularly after a hot bath or shower (aquagenic pruritus) and may be the earliest symptom. The face may be plethoric and the retinal veins engorged. The palms and feet may be red, warm, and painful, sometimes with digital ischemia ( Erythromelalgia ). Over 30% of patients have splenomegaly. Thrombosis may cause symptoms in the affected site (eg, neurologic deficits with stroke or transient ischemic attack; leg pain, swelling, or both with lower extremity thrombosis; unilateral vision loss with retinal vascular occlusion). Bleeding, typically from the gastrointestinal tract, occurs in about 10% of patients. Hypermetabolism can cause low-grade fevers and weight loss and suggests progression to secondary myelofibrosis, which is clinically indistinguishable from Primary Myelofibrosis (PMF) but has a better prognosis. 