Polycythemia Vera Treatment in Mumbai, India

Polycythemia vera is a rare type of rare blood cancer. Originating from the bone marrow, it causes the marrow of the bones to make excessive amounts of red blood cells. These red blood cells thicken the blood and slow down its flow, leading to blood clots, heart attack, or a stroke.

Symptoms of Polycythemia Vera include:

• Headaches
• Dizziness
• Double vision
• Blurred vision
• Fatigue
• Weight loss
• Painful and swollen joints
• Shortness of breath
• Burning or tingling sensations in hands or feet
• Unusual bleeding (for instance, bleeding gums or nosebleed)
• Sweating (majorly at night time)
• Redface (typically looks like blushing or sunburn)
• Body itching (especially after spending some time in hot or warm water)
• Bloating or feeling of fullness just after meals or pain in the left side of the upper abdomen caused by an enlarged spleen.

Polycythemia Vera Causes

Polycythemia vera occurs when a gene (either TET2 or JAK2) causes inappropriate blood cell production. Normally, there are three types of blood cells: white, red, and platelets. But in polycythemia vera, the bone marrow makes few of these blood cells in large numbers.

Polycythemia vera is mainly observed when there are too many red blood cells. However, it can also cause a surge in platelets and white blood cells. While polycythemia vera is not normally inherited from parents, the cause of gene mutation remains unknown.

Polycythemia Vera Stages

Symptoms of polycythemia vera develop gradually over several years. It is marked by the following two stages:

• Early stages: There arent many noticeable symptoms in the early stages. A few specific early-stage symptoms include weakness, headache, itchy skin, dizziness, and unusual sweating. Some uncommon symptoms include a red face, frequent nosebleeds, easy bruising, heavy bleeding from minor cuts, bleeding gums, blurred vision, ringing ears, and a burning sensation in the feet and hands.
• Final stage: In this stage, the scar tissues replace the bone marrow in people suffering from polycythemia vera. This condition is called myelofibrosis. Referred to as the spent phase of polycythemia vera, it is rare and found in a few people with the disease. Along with the symptoms of early stages, this stage is characterized by shortness of breath, bone and joint pain, unexplained, enlarged spleen or liver, and a feeling of fullness in the stomach. This stage increases the risk of complications like frequent infections, anaemia, and a higher risk of bleeding.

Polycythemia Vera Tests

After a physical examination, your doctor can prescribe the following tests:

• Complete blood count (CBC): This test checks the number of platelets, red blood cells, and white blood cells. An unusually high count of any of these could signify polycythemia vera.
• Blood smear: This test determines if you are at risk of any other diseases linked to polycythemia vera.
• EPO level: EPO hormones guide the bone marrow in making blood cells. People suffering from polycythemia vera have lesser amounts of EPO.
• Bone marrow biopsy: The biopsy is conducted to ascertain if your bone marrow produces too many blood cells.

Polycythemia Vera Treatment

Theres no cure for polycythemia vera. Therefore, treatment for polycythemia vera aims to ease symptoms, prevent blood clots, lower the production of red blood cells, and prevent further complications. The treatment options include:

• Phlebotomy: The first and common treatment is phlebotomy or blood withdrawals. This procedure is similar to donating blood (inserting a needle in the vein). Withdrawing blood often decreases excess blood cells and the volume of blood.
• Drugs to lower blood cells: To lower the count of red blood cells, you might be prescribed medicines like hydroxyurea (Droxia, Hydrea), Ruxolitinib (Jakafi), Interferon alfa-2b (Intron A), and Busulfan (Busulfex, Myleran).
• Treatments to ease itching: To ease bothersome itching, you may be prescribed antihistamines. If the discomfort persists, your doctor might recommend ultraviolet light treatment.
• Medications for blood vessels and heart: A low dose of aspirin is recommended to keep blood clots in check. Aspirin also relieves burning sensations in the hands or feet. Furthermore, drugs will be prescribed to control high blood pressure, abnormal cholesterol, and diabetes.
• Radiation treatment: This can help prevent bone marrow from producing excess red blood cells and keep blood thickness and flow within normal levels.

Risks Associated With Polycythemia Vera

If you suffer from polycythemia vera, other serious complications are likely to occur if you develop too many blood clots too often. Here are the factors that increase the risk of developing blood clots:

• being over the age of 60
• a history of blood clots
• high blood pressure
• diabetes
• pregnancy
• smoking

Polycythemia Vera Possible Complications

The slow blood flow and the high blood thickness associated with polycythemia vera can cause the formation of blood clots, leading to a heart attack or stroke. Blood clots can also enlarge the spleen and liver, which can cause sudden and intense pain.

The high levels of red blood cells can lead to kidney stones, stomach ulcers, or gout. Slow blood flow restricts oxygen-rich blood from reaching your organs, leading to heart failure and angina (discomfort and pain in the chest).

Furthermore, polycythemia vera may lead to myelofibrosis, a condition in which scar tissue replaces the bone marrow, leading to abnormal growth of bone marrow cells. When these cells grow beyond control, it can lead to acute myelogenous leukaemia (AML), cancer related to the blood and bone marrow.

Polycythemia Vera Preventions

Primary prevention of polycythemia vera is not possible. You can prevent secondary polycythemia by keeping yourself healthy and comfortable during the treatment:

• Avoid activities that deprive your body of oxygen. For instance, avoid mountain climbing, smoking, or living at a higher altitude.
• Do not chew tobacco. Tobacco tends to narrow blood vessels, allowing blood clots to form easily.
• If you have itchiness with warm water, take a cold water shower.
• Do not scratch your skin. Apply lotions to keep them moist.
• Try light exercises like walking to keep your heart in better health and improve blood circulation.
• Indulge in light exercises for legs and ankles to avoid clot formation.

Epidemiology

• Polycythemia vera commonly affects adults above the age of 60, but it can happen to people of all ages.
• The disease is rare in young adults and children.
• Men are at higher risk for developing polycythemia vera than women.

Prognosis

Right now, theres no cure for polycythemia vera, but with the right treatment and lifestyle changes, you can easily manage this disease for several years.