Do you often feel tired, sometimes have a tingling sensation, or just hear a ringing in your ears? Although these may seem like normal things, sometimes there may be another reason behind them. So, the disease we are going to talk about today is Polycythemia Vera (PV) , which is a condition caused by the excessive production of red blood cells in our body. Although this is a bit of a long name, let's understand it simply.
What is Polycythemia Vera (PV)? Simply put...
Simply put, Polycythemia Vera (PV) is a condition where your body, especially your bone marrow (which we call ``Bone Marrow''), produces more red blood cells than it should. Just like traffic jams, when there are too many cars on the road, the flow of blood becomes obstructed. This causes the blood to thicken and travel slower. This increases the risk of blood clots. This can lead to serious conditions like heart attacks and strokes .
Not only this, sometimes symptoms such as itchy skin, ringing in the ears (`(Tinnitus)`), stomach pain, nosebleeds, and blurred vision may also occur.
Polycythemia Vera (PV) is a chronic condition that cannot be cured completely. But don't worry. With good medical treatment, you can control the symptoms and reduce the risk of side effects. Some people also call it `primary polycythemia`, `polycythemia rubra vera`, `erythema` and `Osler-Vaquez disease`.
Is Polycythemia Vera (PV) a cancer?
Yes, this is indeed a type of cancer. But it is not as serious or as fast-spreading as we might think when we hear the word cancer. This is a blood cancer that belongs to a group called `myeloproliferative neoplasm (MPN) . `MPN` refers to a group of diseases that cause the overproduction of various types of blood cells.
The main cause of Polycythemia Vera (PV) is a change (mutation) in a gene in our body. This is not something that we are born with, but something that occurs during our lives for some unknown reason. This disease develops very slowly. It is often diagnosed after the age of 60. This cancer rarely causes direct death. The main risk comes from complications caused by blood clots. Also, in a very small percentage, it can turn into other serious types of blood cancer.
Who is most affected by this situation?
Polycythemia Vera (PV) is a very rare disease. In a country like the United States, it occurs in about 50 per 100,000 people. It is most common in people over the age of 60.
How does this disease affect our bodies?
As we mentioned earlier, Polycythemia Vera (PV) causes the body to produce too many red blood cells. This excess of cells increases the risk of bleeding, bruising, and blood clots. Because the blood thickens and travels more slowly, our body's tissues and organs don't get the oxygen they need.
This also puts a lot of work on our body's spleen. One of the main functions of the spleen is to filter blood and remove old blood cells. When red blood cells increase, the spleen also becomes overworked, which can cause swelling and pain. This is called `(Splenomegaly)`. Over time, this condition can cause various uncomfortable symptoms. Also, PV can cause other secondary conditions.
What are the risks and complications of Polycythemia Vera (PV)?
Although PV is a progressive disease, there is a risk of sudden blood clots due to the overproduction of blood cells. Over time, PV can also cause other secondary conditions. Very rarely, it can develop into a serious cancer.
Blood clots
This is the main and most immediate risk of PV. If a blood clot forms and travels to the heart or brain, it can cause a heart attack or stroke . If it travels to the lungs (Pulmonary Embolism - PE), it can cause high blood pressure in the lungs (pulmonary hypertension) and heart failure. If a blood clot gets stuck in a vein (Venous Thromboembolism - VTE), it can cause tissue death or chronic venous insufficiency. If a blood clot gets stuck in the main blood vessel to the liver (hepatic vein thrombosis), it can cause blood clots in the liver (Budd-Chiari syndrome), jaundice and liver failure.
Secondary conditions
When red blood cells are produced in excess and broken down, the level of `uric acid` in the body increases. This can cause several problems:
- Kidney stones: When uric acid is deposited in the kidneys.
- Gout: A painful arthritis condition that occurs when uric acid builds up in the joints.
- Stomach ulcers: The extra red blood cells also increase the amount of stomach acid. The extra red blood cells cause the body to release a chemical called histamine, which in turn causes the stomach to produce more acid to fight off the infection. People with PV are about four times more likely to develop stomach ulcers (peptic ulcer disease).
Turning to Leukemia
Polycythemia Vera is a cancer that develops in the bone marrow, but it is not usually a life-threatening condition. With proper treatment, it can be managed well for years. However, in some people, PV can, although rarely, develop into a serious blood cancer called `Acute Leukemia`.
Stages of Polycythemia Vera (PV) Disease
PV disease progresses as follows:
- Early stage (`Early PV`): During this stage, there are few or no symptoms.
- Advancing PV: As PV becomes more severe, you may start to experience more uncomfortable symptoms, or secondary conditions may develop.
- Spent phase: This is called the “spent phase.” At this point, the abnormal blood cells that cause the disease multiply out of control and completely block the bone marrow, which makes blood cells. When these abnormal cells die, scar tissue forms in their place. When most of the bone marrow is filled with this scar tissue, it is unable to make healthy blood cells. Ironically, this causes anemia – a lack of healthy red blood cells. It also increases the risk of bleeding.
Other blood diseases that can occur after PV
When PV becomes severe, it can lead to other blood diseases.
- Myelofibrosis (MF): There is not much difference between the ``spent phase'' of PV and ``Myelofibrosis (MF)'' , another type of blood cancer. Some doctors consider them the same. ``Myelofibrosis'' is when abnormal cells form scar tissue in the bone marrow and fill it. These abnormal cells can spread outside the bone marrow and into other parts of the body. ``MF'' is also a ``myeloproliferative disorder.'' About 10% of ``MF'' patients develop ``Acute Myeloid Leukemia.''
- Myelodysplastic Syndrome (MDS): Very rarely, PV can develop into a condition called Myelodysplastic Syndrome (MDS) . This is a condition in which blood cells do not mature properly, grow abnormally, and die quickly. The body is unable to make healthy, mature cells, resulting in a decrease in the number of different types of blood cells. MDS is more severe than PV or MF, and has a 30% risk of developing Acute Myeloid Leukemia (AML).
- Acute Myeloid Leukemia (AML): About 3% of people with Polycythemia Vera develop Acute Myeloid Leukemia (AML) within 10 years of diagnosis. AML is a serious blood cancer that starts in the bone marrow and can quickly spread to the blood and then to other parts of the body. It requires prompt treatment, but the chances of survival are high.
What are the symptoms of Polycythemia Vera (PV)?
Symptoms often appear very slowly, and sometimes there may be no symptoms at all. First come the vague symptoms common to many diseases:
- Headache.
- Dizziness.
- Fatigue.
- High blood pressure.
- Blurred vision or double vision.
- Hearing a ringing in the ears (`(Tinnitus)`).
Over time, more specific symptoms may appear:
- Sweating, especially at night.
- Difficulty breathing, especially when lying down.
- Itchy skin, especially after bathing in hot water. (This is a somewhat specific symptom of PV)
- Redness, heat, numbness, or burning in the limbs.
- Excessive bleeding or bruising.
- Bleeding from the nose and gums.
- Being thin for no reason.
If PV becomes severe and progresses to secondary conditions, related symptoms may also occur. For example:
Symptoms of Splenomegaly:
- A pain like something is stuck in the upper left part of the abdomen.
- A stomach-filling experience.
- Even after eating a little, I feel full.
Symptoms of stomach ulcers (`Peptic ulcer disease`):
- Pain that comes with a stomach ache.
- Heartburn.
- Acid reflux.
Symptoms of Gout:
- Painful swelling of the joints.
- Joint stiffness.
- Swelling of the big toe.
Symptoms of kidney stones:
- Pain in the lower abdomen or flank.
- Pain when urinating.
- Frequent need to urinate.
Symptoms of Budd-Chiari syndrome:
- Swollen liver, pain in the upper right side of the abdomen, and bloating.
- Yellowing of the eyes and skin (jaundice).
- Ascites and Edema.
Symptoms of Deep Vein Thrombosis (DVT):
- Swelling in an arm or leg and pain when touched.
- There is redness or heat in that area.
- The appearance of bulging blood vessels on the surface of the skin.
Symptoms of a blood clot in the lungs (Pulmonary Embolism - PE):
- Sudden chest pain.
- Shortness of breath.
- Heart rate increases.
Symptoms of Anemia (in the late stages of PV disease):
- Dizziness (vertigo).
- Pale skin.
- Fatigue.
What causes Polycythemia Vera (PV)?
Polycythemia Vera (PV) starts in the soft, spongy part of our bones called the bone marrow. This is where new blood cells are made. PV starts with a single gene mutation in a single stem cell in the bone marrow. More than 90% of the time, this mutation is in a gene called JAK2.
This mutated gene tells the stem cell to keep reproducing. Every cell that is produced continues to replicate itself. Eventually, these abnormal cells fill up the bone marrow, leaving no room for the normal cells.
In most cases, this `JAK2` gene mutation is not something we inherit. It is something that occurs during life, for unknown reasons. However, very rarely, there have been reports of several family members developing PV.
How is Polycythemia Vera (PV) diagnosed?
The World Health Organization (WHO) has proposed three main criteria for diagnosing Polycythemia Vera (PV). A doctor must confirm all three criteria.
Criterion 1: A blood test showing a high red blood cell count:
Red blood cells can be measured:
- High hemoglobin levels (a protein found in red blood cells).
- A high hematocrit level (the percentage of red blood cells in the blood).
- High blood volume (red cell mass).
Criterion 2: Bone marrow biopsy showing one of the following:
- Too many blood cells in the bone marrow, or
- Excessive presence of mature megakaryocytes (cells that make blood platelets).
Criterion 3: The third criterion can be met by showing either of these two:
- Confirmation of the presence of the JAK2 gene mutation through molecular testing, or
- A blood test confirms that the level of `erythropoietin' (a hormone that stimulates the production of red blood cells by the kidneys) in the blood is very low.
"A doctor will look at all of these to tell you exactly whether you have Polycythemia Vera or not. So if you have any symptoms, it's best to see a doctor and get advice."
How is Polycythemia Vera (PV) treated?
In the early stages of PV, treatment is relatively simple. Common treatments include:
Phlebotomy (bloodletting)
This is the main and most common treatment for PV. It is done in the same way that you would donate blood. A healthcare provider inserts a needle into a vein in your arm and removes a small amount of blood (usually about a pint, but the amount can vary depending on your condition). This reduces your total blood volume and the number of extra blood cells.
Low-dose aspirin
This is a medicine that you can buy at the pharmacy. It is usually prescribed to reduce the risk of blood clots. When taken in a low dose once a day, aspirin prevents blood platelets from sticking together. It also helps reduce symptoms such as swelling in the limbs. However, it is a bit harsh on the stomach and increases bleeding. So this is not a good idea if you have stomach ulcers.
If your symptoms are severe, or if you have a history of previous blood clots (thrombosis), you may be placed in a high-risk group and other treatment options may be suggested. Some of these include:
Treatments to reduce itching:
When PV becomes severe, a doctor may prescribe medications like these to treat the itching:
- Antihistamines: These are common medications given for allergies.
- Phototherapy: A treatment that combines ultraviolet (UVA) light with a chemical called psoralen (an organic compound that sensitizes the skin to light therapy).
- SSRIs (Selective Serotonin Reuptake Inhibitors): These are a type of medication typically prescribed for depression. However, when given in very low doses, they have also been found to be effective for persistent physical symptoms such as itching.
Drugs that reduce red blood cell count:
These can be used alone or in combination with each other. Examples:
- `Hydroxyurea`
- `Interferon alfa`
- `Ruxolitinib`
- `Busulfan`
Clinical trials
These give people the opportunity to try out the latest treatments and contribute to medical research. Some of the drugs currently in the research phase are:
- `Peginterferon alfa-2a`
- `Givinostat`
- `Idasanutlin`
Bone marrow transplant
In some cases, a bone marrow transplant may be recommended. A doctor will make this decision based on several factors, such as the severity of your disease and your body's ability to heal.
Supportive care
If your PV continues to get worse despite treatment, your doctor will focus on managing your symptoms. In the later stages of PV, you may experience anemia and an enlarged spleen. You may be given treatments such as:
- Painkillers.
- Blood transfusions.
- Low-dose radiation therapy to the spleen.
What is the life expectancy of someone with Polycythemia Vera (PV)?
Recent studies suggest that the average life expectancy after diagnosis of Polycythemia Vera (PV) is about 20 years. The average age of death is about 77 years. The leading cause of death is complications from blood clots (about 33%). The second leading cause is cancer (about 15%).
"Don't be scared by these statistics. These are normal values. If you get the right treatment and make a lifestyle change, you can stay healthy for a long time."
How can I take care of myself and manage my symptoms?
If you are living with Polycythemia Vera (PV), it is important to stay in regular contact with your doctor. This will help your doctor monitor how your condition is progressing. You can also tell your doctor how you are responding to treatment and whether you are experiencing any side effects. You can live a long time without serious symptoms.
In addition to regular tests and treatment, your doctor will also suggest some simple lifestyle changes that can help prevent blood clots and other complications. For example:
- Exercise. Even a little exercise can help improve blood circulation.
- Avoid smoking. Smoking narrows your blood vessels.
- Avoid environments with low oxygen levels. High altitudes can cause low blood oxygen levels.
- Control your blood pressure. Maintaining a healthy weight will help with this.
Although Polycythemia Vera (PV) is a type of cancer, many people live well for decades after being diagnosed with the disease. This cancer occurs because cells are produced out of control. However, it takes a long time for the uncontrolled production of red blood cells to start affecting you. Even if you do, you can manage the condition for a long time by removing some of your excess blood.
The biggest threat from Polycythemia Vera (PV) is not the cancer itself, but the risk of blood clots. If you're living with PV, be sure to see your doctor regularly. And don't forget to take care of yourself with the "little things" - that is, healthy lifestyle habits. These help maintain the general health of your cardiovascular system, which may be more important than any medical treatment for Polycythemia Vera.
What is the message we want to take home from this story?
Okay, so we've talked a lot about Polycythemia Vera (PV). Here are some of the most important things to remember:
- PV is a blood disease in which the body produces too many red blood cells and grows abnormally. It belongs to a group of cancers called `myeloproliferative neoplasm (MPN)`.
- The main risk comes from blood thickening and blood clotting, which can lead to serious conditions like heart attacks and strokes.
- Symptoms may include itchy skin (especially after bathing), feeling lethargic, headaches, and dizziness.
- Although it cannot be cured completely, it can be managed well with proper treatment, lifestyle changes, and medication. Phlebotomy (bloodletting) and low-dose aspirin are the primary treatments.
- The `JAK2` gene mutation is often the cause of this.
- If you have any of these symptoms, be sure to see a doctor for advice. Early detection increases your chances of avoiding complications and living a healthy life.
Don't be afraid, but don't take it lightly either. Take care of your health. Talk to your doctor regularly, get the necessary tests and treatments on time. Then you can stay healthy even with this condition!
` Polycythemia Vera, Red Blood Cells, Blood Clots, MPN, JAK2, Phlebotomy, Cancer
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