Polycythemia Vera

Disease information

Overview
Causes
and Symptoms
Treatment

Overview

Polycythemia Vera, or PV, is a type of blood disorder known as a myeloproliferative neoplasm. In this condition, your body produces an excess of red blood cells, disregarding the normal regulation mechanisms. This relatively rare disease affects between 0.01 to 2.8 people per 100,000 annually, with the average age at onset being around 60 years old. It's slightly more common in men, with a ratio of about 1-2 men for every woman. In terms of prognosis, the median survival time for all PV patients is over 13 years, and for those under 60 at diagnosis, it extends to around 24 years. The majority of deaths associated with PV are due to complications like blood clots, heart diseases, and secondary cancers. Unfortunately, about 20% of patients may see their PV progress to a more severe condition, such as myelofibrosis or acute leukemia.

Causes and Symptoms

The exact cause of Polycythemia Vera is not yet fully understood. A mutation in the JAK2 gene is found in 95% of patients, and this genetic mutation is not hereditary but acquired, and the exact cause of the mutation has not been identified.

Polycythemia Vera develops gradually, and there may be no symptoms at all for several years. It is often diagnosed through blood tests during regular check-ups before severe symptoms occur. The concentration of red blood cells in the blood increases, making the blood more viscous, and the viscous blood can cause various symptoms because it does not circulate smoothly, especially in small vessels like cerebral vessels.

Enlarged spleen.
Itching that worsens after bathing or showering.
Tinnitus, headache, night sweats, weight loss.
Sudden vision that appears entirely black or seeing something like a black spot.

▲ Itching that worsens after bathing or showering

Treatment

Polycythemia Vera is not a curable disease, and is a condition that requires lifelong management, akin to diseases such as diabetes and hypertension. There are two major complications associated with Polycythemia Vera: the first being thrombosis and hemorrhage, and the second being the progression to acute leukemia and myelofibrosis. As the prevention of complications is closely tied to the patient's survival rate and quality of life, it is paramount to manage the cell count while conducting regular follow-up observations.

Aspirin Used for the prevention of thrombosis.
Phlebotomy Similar to blood donation, this method involves drawing blood from the veins and injecting an equivalent volume of saline to maintain the volume of plasma.
Cytoreductive therapy Hydroxyurea, Peginterferon alfa-2b (Besremi), and Ruxolitinib (Jakafi)