Polycythemia Vera: What Is It?
Polycythemia vera, also known as PV, is a disorder of the bone marrow where too many red blood cells are produced. These cells carry the oxygen around the body and to each of the organs.
The arteries and veins allow the blood to travel around the body but too many red blood cells leads to clotting within them. This is when many of the blood cells group together.
Some of the earlier symptoms are very easy to ignore, especially ones like fatigue. If PV is left untreated, there are serious symptoms and side effects due to the clotting of the blood and these cannot and should not be ignored. The clots mean that the blood is unable to reach various organs, including the brain and back to the heart, causing serious problems for the patient.
There is currently no cure for the condition so the treatment is about preventing the clots from turning into something serious. Patients who choose not to follow the treatment options are likely to die from the clots at an early age due to complication.
Causes of Polycythemia Vera
This is a rare disorder that affects more men than women and is a problem mostly for those over 40. It is linked to the JAK2V617F gene mutation. When the body’s DNA is damaged or changed, which is the reason for fingerprints, eye color and many other physical characteristics, mutations happen.
There is a tight regulation on the blood cell production in the bone marrow and those with PV have too many red blood cells from the bone marrow.
Some of the earlier symptoms are often ignored by patients since they are common physical problems. When not treated, the blood clots do lead to very serious problems.
The earlier symptoms include:
- Difficulty breathing
- Tingling sensations in the feet and hands
Later complications include:
- Deep vein thrombosis (DVT)
- Heart attack
It is possible for doctors to know nothing about PV until there has been a fatal complication. These include:
- Pulmonary embolism – a clot within the lung
- Ischemic stroke – loss of supply of blood to the brain
- Hemorrhagic death – death by bleeding usually from within the digestive tract or stomach
Your doctor will want to order a complete blood count (CBC) test if PV is suspected. This measures how many white and red blood cells are in the blood, along with the hemoglobin levels (the oxygen-carrying protein) and the space the red blood cells take up. Those with PV will find that the red blood cells take up a lot of space.
This is not a condition with a cure. Treatment is all about managing and preventing it from getting worse. Doctors will asses a patient to determine the type of treatment necessary, based on the risk of them developing blood clots.
Those with a low risk of developing clots are:
- Those under 60 and have no blood clotting history
- Those with low cholesterol and blood pressure
Treatment options for low-risk patients include low-dose aspirin and regular phlebotomy. The phlebotomy is when the blood is removed through a vein, usually on a daily basis, until a regular blood count comes back. It is a long process of waiting and seeing what the results say. The aspirin can help with thinning the blood to avoid clots from happening. Low-dose options are also good for reducing the risk of stroke or heart attack.
Patients with a high risk of clotting including:
- Those with a history of clotting
- Those with high blood pressure
- Those with diabetes
There is a more specialized treatment for high-risk patients, using interferon alpha or hydroxyurea.
Interferon alpha is expensive and there is a high risk of flulike symptoms and fever but it is effective in preventing clots. Those with mental disorders/thyroid problems will not be given this medication.
Hydroxyurea reduces the clotting risk by suppressing the amount of red blood cells produced. The downside is the high risk of leukemia developing.
The Future for PV Patients
This is a chronic disorder and the treatments will only decrease the red blood cell production from the bone marrow. It helps to lower the risk of blood clots but cannot completely treat the condition. As long as treatment is followed, it is possible for patients to live without having any complications.