What is Polycythemia Vera?

Polycythemia vera can be described as a rare clinical disorder characterized by the excess production of blood cells by the bone marrow due to cancer leading to too many red blood cells, which then thickens the blood, reducing its flow and causing blood clots and hence resulting in a lot of dysfunction in the blood system.  Picture Courtesy: Shutterstock They usually grow slowly during the years without showing symptoms and are sometimes noticed when screening for another condition. It affects anybody of any age group, but they are very prevalent amongst men.  Picture Courtesy: Osmosis What are the risk factors associated with Polycythemia Vera? The risk factors of polycythemia can occur at any age. But men between the ages of 50 to 70 are at a greater risk than younger men, and younger women are at a greater risk than older women.

What are the Complications associated with Polycythemia Vera?

The complications of polycythemia vera include:

• • • Formation of a blood clot.

The formation of blood clots is due to blood thickness and abnormalities in the platelets. These blood clots could lead to clinical conditions such as stroke, heart attack, blockage in an artery or veins in the abdomen, or leg muscle.

• • • Enlargement of the spleen. 

The spleen helps protect the body by fighting infections and filters unwanted materials which could damage the blood cells. The polycythemia vera increases the number of damaged cells and unwanted materials.

• • • An increase in red blood cells could result in sores on the inner lining of the stomach, intestine, or esophagus called peptic ulcers, inflammation of the joints called “gout”.
    • It could lead to the progressive generation of the bone marrow leading the bone marrow to become scar tissue, which affects the quality of blood cells produced.
    • Pulmonary embolisms may occur.
    • Formation of deep vein thrombosis.
    • Cancer of the bone marrow is called acute leukemia.

 Diagnosis of Polycythemia Vera

The diagnosis of polycythemia vera can be ascertained through:

• • • Blood Test

The patient has to undergo a few blood tests to ascertain the level of their blood cells, such as a hematocrit test, packed cell volume count, etc. The blood cell test will show an increase in red blood cells, white blood cells, and platelets. The blood smear might be taken to the lab, and the blood sample is seen through a microscope. It’s also used to check for other clinical conditions sometimes associated with Polycythemia Vera. Elevated levels of iron-rich proteins are seen.

• • • Hemoglobin Test

A test to check for the hemoglobin levels in the blood.

• • • Bone Marrow Aspiration or Biopsy

This can be used to determine polycythemia vera. This procedure is carried out with the doctor inserting a needle into the hip bone, and a sample of the bone marrow is collected and taken to the lab.  When the liquid sample is drawn, it is called a bone marrow aspiration, but when a solid part of the bone marrow is taken as a sample, it is called a bone marrow biopsy. Picture Courtesy: shutterstock

• • • Gene Testing

Specific genetic testing can be carried out to diagnose polycythemia vera. It aims at showing the gene that has the mutation or if there Is any mutation.

• • • Complete Blood Count Test

This is a procedure whereby a sample is taken from the blood and sent to the lab, where a machine counts the number of red blood cells, white blood cells, and platelets to check for an abnormal increase of blood cells.

• • • Erythropoietin Level Test

This test ascertains the level of erythropoietin hormone in the blood. Erythropoietin is responsible for signaling the bone marrow to make blood cells. In the cases of People with polycythemia vera, erythropoietin is very low.

How to treat polycythemia vera?

There is no cure for polycythemia vera. The main aim of the treatment is to focus on reducing the risk of complications and ease the symptoms of the patient.

• • • The doctor administers medications containing antihistamines to reduce itching.
    • The use of blood withdrawal technique. This technique involves withdrawing blood from the body by inserting a needle into a vein (phlebotomy). It is also used to donate blood in the lab.
    • The doctor administers medications that will relieve or treat depression. They are called selective serotonin reuptake inhibitors (SSRIs). They include paroxetine (Brisdelle, Paxil, Pexeva, others) or fluoxetine (Prozac, Sarafem, Selfemra, others).
    • Medications to reduce blood cell production.
    • Heart medications such as low dose aspirin are administered to prevent clotting of blood vessels of the heart. The low dose aspirin is also used to reduce the burning pain of the hands and feet.
    • The doctor also prescribes medications to help control diabetes, abnormal cholesterol, and high blood pressure.

Lifestyle and Home Remedies for Polycythemia Vera

A few adjustments in lifestyles and habits could enhance the comfort and deal with the symptoms.

• • • Make a habit of frequently exercising as it could increase the blood flow and decreases the risk of blood clotting. Arms and leg stretches could also help reduce clotting at deep blood vessels in those areas and improve blood flow.
    • Avoid smoking and the use of tobacco. Using tobacco may cause the blood vessels to constrict. As a result, increasing the chances of blood clotting, which could lead to stroke, heart attack, and even death.
    • Try spending time in places that are ventilated.
    • Avoid bathing in hot waters. Bath more often with cold waters to avoid triggering the itching.
    • Try not to scratch the skin during the itching as this might damage the skin.
    • Try staying away from extreme conditions such as getting exposed to too hot or too cold conditions.
    • Always keep hydrated.
    • Avoid stimulants such as alcohol, chocolate, coffee, tea, use of Laptops, phones, etc., before sleeping.

What is the recovery and survival rate of polycythemia?

People with polycythemia vera’s recovery and survival rate are approximately 1.5 to 3 years without proper treatment and management.  With treatment and the emergence of new technologies in the medical field, it is an estimated 14 to 24 years. This is most accurate for people, mostly men, under 60 years. What are the food items that have to be avoided by people who are diagnosed with polycythemia vera? Picture Courtesy: wellhealth There are certain foods to avoid if a person has been diagnosed with polycythemia, including sugar, carbohydrate-rich foods, fatty foods, and refined and processed foods. Try eating foods rich in vitamins such as vegetables, legumes, etc. Drink lots of water to stay hydrated. Staying hydrated helps prevent the thickening of your blood.  

Symptoms

What are the most common symptoms associated with polycythemia vera?

The symptoms of polycythemia are hardly visible and noticeable till a period. They include:

• • • Headaches
    • Dizziness and confusion
    • Numbness of hands and feet
    • Distorted and blurred vision
    • Tingling and burning sensations in hands and feet
    • Enlargement of the spleen
    • Unusual bleeding, especially at the nose and gum
    • Itching, especially after a warm bath
    • Pains in the abdomen, especially in the upper left region.
    • Swellings at the joint, especially at the toe
    • Shortness of breath, especially when lying down
    • Easily satisfied or feeling of fullness
    • Having double vision
    • Dark or blind spots in the vision come and go.

Causes

What are the most common causes of polycythemia vera?

Polycythemia vera is caused mainly by a gene mutation that is responsible for the signaling of the production of blood cells in the bone marrow where the blood cells are produced. 

The blood cells include red blood cells, white blood cells, and blood platelets, these mutations lead to excess production of the blood cells, slowing blood flow, and blood clots are easily formed as a result.

FAQ

• • • Does Aspirin lower red blood cell count?

Aspirin has been found to help reduce red blood cell count, hemoglobin count, and other cell indices. Aspirin also affects platelet rate in the blood, reducing the risk of developing blood clots.

• • • Can polycythemia vera kill?

Polycythemia, when left untreated, has serious complications and can be quite fatal and hamper the normal body functioning by leading to pulmonary embolism, deep vein thrombosis, heart attack, stroke, and possible death.

• • • Is polycythemia cancer?

Polycythemia is a cancerous blood disorder. Here, the blood cells are produced in excess by the bone marrow, making the blood thicker and increasing the risk of blood clotting.

• • • Is polycythemia a disability?

Polycythemia could bring limitations to one’s lifestyle and comfort without proper treatment and management, but one can reduce the severity with proper management and healthy habits.

• • • How fast does polycythemia progress?

Polycythemia is a rare blood cancer that progresses slowly without visible signs or symptoms. A recent study shows that 2% to about 14% of cases indicate that polycythemia becomes fully cancerous within 10 years of its first inception.