Polycythemia Vera: Causes, Symptoms and Treatments

Overview

Polycythemia vera is a rare blood cancer which causes the bone marrow to produce too many red blood cells. The increase in red blood cells thickens the blood and slows down the blood flow, causing severe health issues, including blood clots.  

It is a rare form of cancer that typically develops gradually and is often diagnosed after you are 60 years. Typically, the condition is diagnosed during a routine blood test for another health issue or reason. Failure to get proper medical treatment can be fatal. However, timely treatment can reduce signs, symptoms, and complications.

The blog explains polycythemia vera, its types, causes, diagnosis, and treatment options. 

What is Polycythemia Vera?

Polycythemia vera (PV) is a rare blood disorder that develops in the bone marrow. Bone marrow is the soft center that is present in your bones. It is responsible for making new red blood cells. However, when you have PV, it can make excessive red blood cells , makes  your blood thicker and sluggish, resulting in the risk of blood clots, heart attacks, stroke, and other complications. 

The condition doesn’t have a cure and is a slow-progressing disease. But with timely and correct medical care, your doctor can manage your symptoms and any risk of complications. Most PV patients do not get diagnosed till the age of 60 years. The diagnosis is typically accidental as doctors may be investigating other health conditions. It affects men more than women. 

Polycythemia vera is also known as primary polycythemia, polycythemia rubra vera, erythema, and Osler-Vaquez disease. 

What are the Symptoms of Polycythemia Vera?

Some people do not experience any signs or symptoms. However, over time some may experience vague symptoms, such as:

• Headaches
• Dizziness 
• Fatigue
• Ringing in the ears
• Blurred or doubled vision
• Increased blood pressure 

However, as the disease progresses, the symptoms also become a little more severe, including: 

• Itchiness, particularly after a warm bath or shower
• Numbness, tingling, burning, or weakness in the hands, feet, arms, or legs
• A sense of fullness soon after eating a meal and bloating or pain in the left upper abdomen due to an enlarged spleen
• Unusual and unexpected bleeding, including nosebleeds or bleeding gums
• Painful swelling in one joint, mainly in the big toe
• Breathing problems when lying down
• Excessive sweating, particularly at night
• Unexplained weight loss
• Gout
• Easy bruising
• Concentration issues 
• Flushed face

When to Seek Medical Help?

Contact your doctor immediately if you notice signs and symptoms of polycythemia vera disease.

Request an appointment at Apollo Hospitals               

What are the Causes of Polycythemia Vera?

The cause of polycythemia vera is a mutation of the JAK2 gene. The gene controls the protein that helps the bone marrow produce blood cells. Nearly 95% of cases of PV patients have these mutated genes. However, these mutated genes are not usually inherited  from your family members. Experts are working towards finding a cause for the mutation of the gene.

What are the Ways Doctors Diagnose Polycythemia Vera?

After taking your detailed medical history and performing a physical exam, your doctor may recommend the following tests:

• Blood tests measure the levels of red blood cells. The test may show higher levels of red blood cells, increased red blood cells in total blood volume, and elevated levels of an iron-rich protein that carries oxygen. 
• Bone marrow biopsy: Your doctor may collect a sample of your bone marrow to check if your bone marrow is producing increased red blood cells .
• Gene testing: If your doctor suspects polycythemia vera, your bone marrow or blood may be used to test for a mutated gene associated with the disease. It can also show decreased levels of erythropoietin – a hormone produced by the kidneys to promote red blood cell production. 

What are the Various Treatment Options?

As mentioned earlier, the disease is incurable. However, there are treatment options that relieve symptoms and reduce the risk of complications. The following are the treatment options that your doctor may recommend:

1. Phlebotomy: It is most commonly known as blood withdrawals. It is the same as donating blood. The procedure reduces the excess blood cells. The frequency of the treatment course depends on the severity of your condition. 
2. Medications: There are several medications prescribed to reduce various symptoms. Antihistamines and selective serotonin reuptake inhibitors like fluoxetine, or recommended ultraviolet light treatment, relieve bothersome itching. Drugs such as hydroxyurea, interferon alfa-2b, ruxolitinib, and busulfan, help reduce the number of red blood cells in the bloodstream. Sometimes, your doctor may prescribe medicines to control heart and blood vessel disease risk factors, such as high blood pressure, diabetes, and abnormal cholesterol. Your doctor may prescribe a low dose of aspirin to reduce or prevent blood clots from forming. 
3. Bone marrow transplant: Sometimes, your doctor may recommend a bone marrow transplant. It depends on several factors, such as the severity of the condition and your body’s healing capacity. 

Conclusion

Polycythemia vera is a rare form of cancer that is incurable. However, many patients have lived for a long time after diagnosis. You can manage the disease with timely treatment and prevent various health complications. Healthy lifestyle changes also help in managing your condition. 

Frequently Asked Questions (FAQs)

1. What are the complications of polycythemia vera?

The following are the complications:

• Heart attack
• Stroke
• Pulmonary embolism 
• Enlarged spleen and liver

2. Who is at risk of developing the disease?

It is common in older adults above the age of 60 years. 

3. What are the lifestyle changes that help manage the disease?

Potential polycythemia vera complications of include:

1. Blood clots: Decreased blood flow and increased blood thickness, as well as abnormalities in the platelets, raises the risk of blood clots, which can lead to a heart attack, a stroke, or a blockage in an artery in the lungs or a vein in the abdomen or deep within the leg muscle or.
2. Enlarged spleen: The raised blood cells due to polycythemia vera makes the spleen work harder than normal, causing it to enlarge.
3. Problems due to high levels of red blood cells. Too many red blood cells can cause a variety of other complications, such as open sores on the inside lining of the stomach, upper small intestine or esophagus (peptic ulcers) and inflammation in your joints (gout).