How Treatment Improves Polycythemia Vera Life Expectancy

Polycythemia vera is a medical condition that causes the body to make too many red blood cells. It is classified as a type of cancer. The excess red blood cells can cause dangerous complications such as blood clots, but the red blood cells do not invade healthy tissue. Polycythemia vera can progress to myelofibrosis or acute myeloid leukemia, which are aggressive types of cancer.

This condition can be treated with medical interventions and medication to reduce the effects of complications and improve life expectancy. Since there is no cure, disease surveillance and treatment have to be continued long term.

This article will discuss the life expectancy for polycythemia vera with or without treatment, and what treatments may be offered.

What Is Polycythemia Vera? 

Polycythemia vera is caused by an abnormality in the red blood cell production that begins in the bone marrow. Secondary polycythemia is a similar condition that occurs due to an underlying disease. Both conditions cause the body to make too many red blood cells, but secondary polycythemia is not considered a type of cancer.

Polycythemia vera is defined by:

• Hemoglobin (the oxygen-carrying molecule in red blood cells) is higher than 16.5 grams/deciliter (g/dl) for males (normal is 13.2–16.6 g/dL) or higher than 16.0 g/dL for females (normal is 11.6–15 grams/dL).
• Hematocrit (the percentage of blood that is red blood cells) is higher than 49% for males (normal is 38.3%–48.6%) or higher than 48% for females (normal is 35.5%–44.9%).
• Mutation in the gene that codes for the Janus kinas 2 (JAK2) protein may be present.
• Characteristic changes are seen in the red blood cell precursors (substances from which red blood cells form) in the bone marrow.
• Erythropoietin, the hormone that stimulates red blood cell production, may be elevated.

Secondary polycythemia can occur due to medical conditions that cause the body to make too many red blood cells. These include heart disease, lung disease, sleep apnea, and some cancers. 

Polycythemia Vera Life Expectancy

Polycythemia vera can lower a person’s life expectancy due to complications, including blood clots that can affect the heart, brain, lungs, or other organs. These effects may cause lasting disability and can be fatal. Survival rates have been increasing due to improved treatments.

Secondary polycythemia is associated with an increased risk of death as well.

Without Treatment 

If you are not treated for polycythemia vera, it can lead to death within months or years, but you can survive for longer. The exact survival with polycythemia vera without treatment is not known. A person may survive for many years if complications do not occur. Sometimes, even with specific treatment to manage complications, the complications can be fatal.

It is not possible to predict whether a person who is diagnosed with polycythemia vera will develop complications, when they may occur, or the severity and effects of the complications. 

With Treatment 

Treatment prolongs survival because it can help prevent serious complications of the condition. Interventions can help prevent complications from happening and may help reduce the severity and frequency of complications. Complications of polycythemia require their own treatment.

With treatment, the median survival rate is approximately 15 years after diagnosis, and for patients aged 40 or younger, median survival is 37 years after diagnosis. 

After a Polycythemia Vera Diagnosis 

If you are diagnosed with polycythemia vera or secondary polycythemia, you may need to start treatment. The treatment will depend on your red blood cell values, your symptoms, your risk factors for complications, and any complications that you have already experienced. 

Treatments include:

• Phlebotomy is used to remove blood from the body Like a blood donation, a line is inserted into a vein and blood is collected in a blood collection bag. This procedure may need to be repeated at regular intervals or when the red blood cell count increases.
• Aspirin is used to prevent blood clots.
• Medications include Droxia (hydroxyurea), Multiferon (interferon alpha), and Jakafi (ruxolitinib phosphate), a JAK inhibitor. used to inhibit the production of red blood cells.

If you have secondary polycythemia, the treatment is focused on the management of the causative primary disease. In some instances, phlebotomy is necessary for the treatment of secondary polycythemia as well.

Continuing With Treatment 

Treatment needs for polycythemia vera may change over time. You will need to have your red blood cell count, hemoglobin, and hematocrit measured periodically to determine which treatments you should continue. 

Summary 

Polycythemia vera and secondary polycythemia are conditions in which the body makes too many red blood cells. These conditions need to be treated because they can cause serious complications and may affect survival. With treatment, life expenctancy is substantially improved.

Polycythemia vera is treated with phlebotomy (removal of blood), aspirin to prevent blood clots, and medications to reduce red blood cell production. Secondary polycythemia requires management of the underlying condition and sometimes phlebotomy.

A Word From Verywell

Living with polycythemia vera or secondary polycythemia means that you have to be attentive to your health and your medical care. Treatment and surveillance of the effects of your disease must be consistent and lifelong. While polycythemia can present challenges, treatment will help improve your quality of life and life expectancy.