Everything You Need to Know About Polycythemia Vera

Polycythemia Vera: What Is It?

Polycythemia vera (PCV) is a genetic disorder caused by too many copies of one or two genes. A person with PCV will have at least three normal blood cells, but only one or two normal red blood cells. These abnormal red blood cells are called erythrocytes and they carry oxygen around your body. If there are too few red blood cells, then the person cannot get enough oxygen to all parts of their body. If there are too many red blood cells, then they may suffer from organ failure. People with PCV usually develop symptoms between ages 10 and 30 years old.

Symptoms of Polycythemia Vera

The most common symptom is fatigue, which occurs when the body does not produce enough energy due to low levels of oxygen in the bloodstream. Other symptoms include shortness of breath, weakness, muscle pain, and numbness.

Polycythemia Vera Treatment Options

There are no known treatments for polycythemia vera. However, doctors can monitor the condition using tests such as blood counts and other lab work. There is also a drug called methotrexate that has been used successfully to treat some cases of PCV.

What Are the Stages Of Polycythemia Vera?

The disease is broken up into three stages.

Many patients experience a very mild version of PCV, which has few or no symptoms. This is known as monoclonal gammapathy of undetermined significance, or MGUS.

While the disease is usually diagnosed at stage 1, some people progress to the next stage within one year. In these cases, the patient’s spleen and liver begin to malfunction as a result of having too many red blood cells. This is known as accelerated phase polycythemia vera.

During stage 2 or 3, patients develop organ failure and suffer severe anemia.

In rare cases, people live with PCV for years or decades. In other cases, the disease progresses quickly and proves fatal.

How Long Does Someone With Polycythemia Vera Live?

Life expectancy for someone with PCV is generally between two and five years after diagnosis. However, some people live with the disease for 10 to 20 years or longer.

Polycythemia Vera Is Not Cancer: What’s the Difference?

We often hear about different types of cancer in the news, such as lung cancer and skin cancer.

But what exactly is cancer?

Cancers start when cells begin to divide and grow out of control. As the cancer cells continue to grow, they may start to invade and damage neighbouring organs. The problem usually starts in a single part of the body, such as the lungs or skin, but it may eventually spread to other areas.

Cancers are usually described by where they start in the body or by their appearance under a microscope. For example, cancers that begin in the lungs are called lung cancer. Cancers that begin in the skin are called skin cancer.

In contrast, PCV does not involve abnormal cell division. Instead, it affects the number of red blood cells in your body.

It’s also important to understand that PCV is not contagious. This means it is not spread from one person to another. Instead, the disease is genetic. This means that if someone has the genes for PCV, they may develop it, even if all their immediate family members have never had it.

Diseases Often Linked to Polycythemia Vera

Having PCV is not the only thing that can cause diseases. Other factors can also increase your risk of developing a related condition. One common example is smoking. People who smoke are more likely to develop lung cancer and other diseases because of the chemicals in cigarettes.

In a similar way, someone who has PCV is more likely to develop other related conditions. These conditions are most commonly linked to blood disorders and complications of the spleen.

The most common related condition is myelodysplastic syndrome. This causes anemia, or a lack of red blood cells. The spleen also becomes enlarged and no longer functions properly, leading to a shortage of infection-fighting white blood cells. People with myelodysplastic syndrome are also at risk of developing leukemia.

If you have PCV and develop any of these conditions, talk to your doctor about your specific risk factors. They may be able to advise you about changing your treatment or helping to prevent complications.

If you have any further questions on what is PCV or its treatment options, speak with your doctor.

What Causes Polycythemia Vera: Causes and Types

The exact cause of PCV is not yet known. Doctors do know that it is not contagious, so it cannot be transmitted from one person to another. This means that it is not caused by a virus or bacteria, but by a problem with your genetics. It’s very rare to have this disease.

The exact type of genetic problem is also unknown. Doctors do know that it tends to run in families, and if someone in your family has been diagnosed with it, you may be more likely to develop it too. There are three main types of PCV that are most common:

Primary. This is the most common type and accounts for around 70% of all cases.

Secondary. This occurs after an infection or injury to the bone marrow.

Tertiary. Also known as hhv.. This is the least common, and it involves the excessive division of blood cells in the liver.

Some of the common symptoms that can occur with this condition are:

Easy bruising or frequent bleeding from cuts.

Shortness of breath or difficulty breathing.

Feeling very tired for no reason.

Severe nosebleeds or bleeding gums.

Bright red urine or blood in your stool.

Fever, chills, cough and weight loss.

If you notice any of these symptoms, be sure to see a doctor immediately. They may want to test you for PCV or other conditions. It’s important that you get treatment as soon as possible.

What Is the Treatment for Polycythemia Vera?

The main treatment options for PCV are based around treating the symptoms when they occur. For example, if you suffer from nosebleeds, your doctor might suggest a prescription medication to reduce the bleeding. Alternatively, they can cauterize or clamp off bleeding blood vessels.

More serious cases of PCV require a transfusion of red blood cells or an infusion of antibodies. In more serious cases, a bone marrow transplant may be necessary. This is only recommended in the most serious and life-threatening cases, however.

The outlook for people with PCV is good if it’s diagnosed and treated at the earliest opportunity. Otherwise, it can lead to more serious complications. Getting regular check-ups from a doctor is important so any problems can be caught early.

The disease is rare but fairly well known. As such, there are many online support groups and communities where people with the condition or their families can meet and offer each other advice and support. Some of these groups also raise awareness of the condition, which may result in earlier diagnosis and better treatment outcomes.

Sources & references used in this article:

From leeches to interferon: should cytoreduction be prescribed for all patients with polycythemia vera? by JJ Kiladjian, T Barbui – Leukemia, 2020 – nature.com

Thromboembolic complications of polycythemia: Polycythemia vera versus smokers’ polycythemia by TH Schwarcz, LA Hogan, ED Endean… – Journal of vascular …, 1993 – Elsevier

Diagnosis and management of polycythemia vera: proceedings from a multidisciplinary roundtable by LA Raedler – American health & drug benefits, 2014 – ncbi.nlm.nih.gov


Characteristics and outcomes of patients with essential thrombocythemia or polycythemia vera diagnosed before 20 years of age: a systematic review by JC Ianotto, N Curto-Garcia, M Lauermanova… – …, 2019 – haematologica.org

Endovascular stent graft repair of thoracic aortic mural thrombus in a patient with polycythemia vera: a word of caution by S Fukuhara, S Tyagi, E Clarke-Pearson, T Bernik – Vascular, 2015 – journals.sagepub.com

Acute coronary artery bypass graft failure in a patient with polycythemia vera by H Osada, H Nakajima, K Meshii… – … and Thoracic Annals, 2016 – journals.sagepub.com

Different effect of hydroxyurea and phlebotomy on prevention of arterial and venous thrombosis in polycythemia vera by T Barbui, V De Stefano, A Ghirardi, A Masciulli… – Blood cancer …, 2018 – nature.com