What Does Polycythemia Vera Mean?
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What Is Polycythemia Vera?

Published on Aug 03, 2022 and last reviewed on Dec 14, 2022   -  5 min read

Abstract

Polycythemia vera is a very rare type of hematological cancer. Let us sweep through the subject in depth below.

What Is Polycythemia Vera?

Introduction:

The increased blood cell level caused by polycythemia vera is due to overproduction in the bone marrow, which is the soft tissue found within the bones. It can lead to elevated hemoglobin and predispose an individual to develop blood clots by making the blood thicker. In most cases, the production of white blood cells and platelets is also high, but the increase in red blood cell production is most noticeable.

The condition is one of several types of myeloproliferative neoplasms, which damage the bone marrow and result in excessive red blood cells, white blood cells, or platelets.

This could cause many life-threatening conditions such as stroke and many other organ damages. Without treatment, living with this condition is hard. Treatments can help ease the symptoms and complications. Treatments include removing blood at regular intervals, medications to lower blood cell formation, and prevention of blood clots.

Patients may progress to myelofibrosis which causes scarring of the bone marrow leading to anemia; individuals may need blood transfusions or more aggressive therapy. It advances slowly and is frequently detected beyond the age of 60.

What Factors Contribute to Polycythemia Vera?

The blood that carries oxygen and nutrients to different body parts is made up of hematopoietic stem cells. In addition to reproducing, hematopoietic stem cells also produce mature blood cells. These stem cells are produced in the bone marrow, which is a spongy tissue. It is only after the blood cells have reached full maturity and are functioning properly that they exit the bone marrow and enter the bloodstream.

But individuals with polycythemia vera produce numerous amounts of red blood cells. This is triggered by a gene (either JAK2 or TET2) that does not function properly. As the mutated cells proliferate, they become the predominant hematopoietic cells in the bone marrow. In most instances, it is unknown why this occurs.

How Many Types of Polycythemia Vera Are Present?

There are two types of polycythemia vera, relative and absolute polycythemia vera.

  • Relative Polycythemia Vera- This is not a true polycythemia vera; what happens here is the plasma volume decreases; thus, it is known as relative polycythemia.

  • Absolute Polycythemia Vera- On the other hand, is true. It is further divided into two classes, which are primary and secondary. In the primary, the erythropoietin level is very decreased, and in the secondary, the erythropoietin increases could be either compensatory or abnormal.

What Are the Symptoms of Polycythemia Vera?

Most of the symptoms are similar to the other blood conditions. As a result, never neglect any indicators of the ailment and seek medical attention right away.

  • Fatigue.

  • Dizziness.

  • Blurred vision.

  • Nosebleeds.

  • Bleeding gums.

  • Budd-Chiari syndrome (a disorder in which a clot blocks or narrows the hepatic veins).

  • Too much menstrual bleeding.

  • High blood pressure.

  • Unexplained weight loss.

  • Redness in the face (the face becomes flushed and red).

  • Increased sweating usually occurs at night.

  • Enlargement of the spleen (splenomegaly) is also common because the excess red blood cells build up in the spleen, which usually helps with removing excess cells.

  • Itchiness can occur after a warm hot shower; this is due to the increased number of basophils and mast cells that contain histamines that cause itching when released.

  • High turnover of the red blood cells can cause an increase in uric acid, which causes inflammation.

  • Stroke and heart attacks occur due to increased blood clots.

  • Deep vein thrombosis (veins on the surface of the skin are enlarged).

  • Shortness of breath and trouble breathing while lying down.

How Is Polycythemia Vera Diagnosed?

  • Routine Blood Test: This can be carried out to find the increased hemoglobin or hematocrit levels, as well as an increased white blood cell and platelet count.

  • Genetic Testing: This could determine the presence of JAK2 gene mutation.

  • Bone Marrow Tissue Examination: This can be done to look for signs of fibrosis and excess production of red blood cells.

Treatment

How Is Polycythemia Vera Treated?

Polycythemia vera is a long-term illness for which there is no solution. However, medication might help in the better management of its symptoms and in avoiding consequences.

  • Phlebotomy- Most individuals require this, which is when the blood is drawn through the vein and removed every few months. This blood is then donated to other individuals, but it can be used for autologous donation; donors can use it in the future.

  • Myelosuppressive Medications- Such as Hydroxyurea and Ruxolitinib, are helpful in relieving symptoms like itching.

  • Other Medications- Such as Antihistamines, Hydroxyzine, and Aspirin, can also reduce inflammation and also helps in preventing the clotting of the blood.

  • Chemotherapy- Among other medicines, it decreases the production of blood cells in the bone marrow. Furthermore, it maintains the blood flow and thickness at a normal level.

What Can Be Done to Manage Polycythemia Vera?

There is no treatment for this condition, but there is always a method to manage it effectively by adopting a few good lifestyle habits such as,

  • Even little exercise might help improve circulation.

  • Tobacco product usage should be avoided. It causes the blood vessels to constrict, resulting in a blood clot.

  • Avoid being in low-oxygen settings. High elevations might lower the amount of oxygen in the blood.

  • Maintaining healthy blood pressure and weight can benefit from this.

  • Eating nutrient-dense and balanced meals rich in fresh fruits and vegetables, whole grains, lean protein, and low-fat dairy will aid in the management of the disease.

  • Legs and ankles stretching can be done.

  • Avoiding high temperatures and protection from the sun is essential. Hot tubs and hot baths of any kind should be avoided since they might harm the skin.

  • Avoid dehydration by drinking plenty of water.

  • High salt diets must be avoided as they might cause the body to move water into the tissues, worsening some of the symptoms.

  • Avoid trauma and circumstances where there is a high risk of getting hurt.

Conclusion:

Ultimately, polycythemia vera is a kind of cancer, but most people continue living well for decades after diagnosis. Because it involves the uncontrolled growth of cells, it is considered cancerous. However, it takes a long time for unregulated red blood cell production to start hurting. When they do, it can be controlled for a long period by getting some more blood drawn.

The most serious concern posed by polycythemia vera is not malignancy but the possibility of blood clots. It is vital to see the doctor regularly if the patient has polycythemia vera. And never overlook self-care in the "small ways" – good lifestyle practices that can help preserve the overall cardiovascular health, which may be just as essential as any medical therapy for polycythemia vera.

In potentially life-threatening situations, the relevance of avoidance and approach reasons should be considered. It mainly affects adults over the age of 60. Medications are given to prevent blood clots and pain mainly. Mostly, the diagnosis depends on blood counts and bone marrow biopsies.

Last reviewed at:
14 Dec 2022  -  5 min read

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