Polycythemia Vera
General Information
Epidemiology of Polycythemia Vera (PV):
- Annual incidence increases with advanced age, varies from 0.7 to 2.6 per 100,000 people in Europe and North America (Hultcrantz M. et al. J Intern Med. 2020; 287: 448-454)
- Slight male predominance (M:F = 2:1) and median age at diagnosis is 61 years (Leukemia 2013;27:1874)
Clinical Picture of Polycythemia Vera
- At the beginning, a person may not notice any symptom, but when symptoms do appear, they include:
- Pruritus, or itchy skin, which is worse after warm showers or baths
- Blurry vision or other changes relating to eyesight
- Headaches
- Excessive sweating
- Fatigue
- Dizziness
- Ringing in the ears
- Clinical signs of the disease may include:
- Splenomegaly
- Skin that appears red or discolored, or rarely erythromelalgia (redness and warmth skin, with burning pain)
- Unexplained weight loss
- GI discomfort and peptic ulcer disease are common
- During the course of disease bleeding and thrombotic complications may be observed:
- Bleeding events can include epistaxis, gum bleeding, and gastrointestinal (GI) bleeding.
- Thrombotic events can include deep venous thrombosis (DVT), pulmonary embolism (PE), Budd-Chiari syndrome, splanchnic vein thrombosis, stroke, and arterial thrombosis
Diagnosis and Prognosis
Differential Diagnosis of Erythrocytosis (xxxx – allegato 1)
Diagnostic Criteria of Polycythemia Vera (PV) according to the WHO and ICC Classifications (allegato 2)
Prognostic Factors and Models in Polycythemia Vera (allegato 3)
Therapies
Absolute indications for initiating cytoreductive therapy in High Risk Polycythemia Vera (allegato 4)
Low-risk PV patients eligible for cytoreduction (allegato 5)
Switching Between Therapies in Polycythemia Vera (allegato 6 o 6b)
Resistance/intollerance
Clinical resistance/intolerance to hydroxyurea in Polycythemia Vera (allegato 7 –> link & Allegato 8)
Intolleranza a breve termine: incidenza e management
Intolerance to Ruxolitinib in Polycythemia Vera (allegato 9)
Intolerance to Interferon in Polycythemia Vera (allegato 10)
