Polycythemia vera: symptoms, causes and treatment

Definition

What is polycythemia vera?

Polycythemia vera is a type of blood cancer that develops in the bone marrow. This disease occurs when the bone marrow produces more red blood cells than normal.

Too many red blood cells can make the blood thicker and cause obstruction of blood flow in the arteries and veins. This condition can cause serious health problems, such as blood clots.

In addition, blood flow that is not smooth can inhibit the distribution of oxygen to all parts of the body, including organs. Thus, organ function will also be disrupted.

Unfortunately, polycythemia vera is not a disease that can be cured. However, with early treatment, this disease can be well controlled and does not cause other health problems.

However, if not treated promptly, polycythemia vera can be life threatening and increase the risk of more severe health complications.

How common is polycythemia vera?

Polycythemia vera is a very rare type of blood cancer. This condition is estimated to only occur in 22 out of 100,000 people in the world. As for other types of blood cancer that are more common, namely leukemia, lymphoma (lymphoma), or multiple myeloma.

This disease affects men more often than women. All age groups can experience this condition, but the case is more often found in the elderly, over the age of 60 years.

Polycythemia vera is also rare in children or people under the age of 20. Talk to your doctor for more information.

Signs & symptoms

What are the signs and symptoms of polycythemia vera?

Polycythemia vera is a disease that develops slowly. Sufferers may not feel any symptoms for years, especially in the early stages.

However, in some people, the signs and symptoms of polycythemia vera that usually appear are:

• Shortness of breath and difficulty breathing when lying down.
• Dizzy.
• Excessive bleeding, such as nosebleeds or bleeding gums.
• Headache.
• Blurry vision
• Excessive sweating at night.
• Weight loss without an obvious cause.
• Itching all over the body, especially after a warm shower, and redness of the facial skin.
• Numbness, tingling, burning, or weakness in the hands, feet, arms, or legs
• Very painful swelling on one alone, often in the big toe.
• A feeling of pressure or fullness on the left side of the stomach due to an enlarged spleen.

There may be other signs and symptoms not listed above. If you have concerns about a particular symptom, do not hesitate to consult your doctor.

When to see a doctor?

If you begin to feel the symptoms above, you should immediately see a doctor. The symptoms above do look like other signs of disease. However, there is nothing wrong with confirming the cause of these symptoms to the doctor to get the right treatment.

The signs and symptoms that everyone feels can be different. Immediately contact your doctor if you feel any changes in your body condition.

Causes & risk factors

What causes polycythemia vera?

Doctors generally divide these diseases into two categories based on their cause, namely:

1. Primary polycythemia

Primary polycythemia is the most common type. This type of polycythemia occurs due to genetic changes or mutations in JAK2.

According to the MPN Research Foundation, 95% of people with polycythemia vera have the problematic JAK2 gene. However, until now it has not been found the exact cause of this gene mutation.

Primary polycythemia is not a condition that is passed from parent to child. However, in some cases, this genetic mutation can run in families.

2. Secondary polycythemia

This type of polycythemia is not associated with a JAK2 gene mutation. This condition is caused by low oxygen levels in the body, especially blood.

If the body is deprived of oxygen for a long time, your kidneys will produce the hormone erythropoietin (EPO). The excess EPO hormone can stimulate the bone marrow to produce more red blood cells than usual.

Some of the diseases that can cause secondary polycythemia include:

• Chronic lung disease (COPD) and sleep apnea

This condition can cause the body to lack oxygen. This can trigger an increase in the production of the hormone EPO and red blood cells in the body.

• Kidney problems

In rare cases, the production of the hormone EPO can also increase if the kidneys are damaged, such as a tumor or narrowing of the blood vessels.

What increases the risk of getting polycythemia vera?

Polycythemia vera is a rare disease that can affect anyone. However, there are several factors that can increase your risk for developing this disease.

Having one or more risk factors does not mean that you will definitely develop this disease. Patients suffering from this disease may also have unknown risk factors.

Here are some factors that can increase the risk of polycythemia vera:

1. Age

People over 60 years of age are at a higher risk of developing the disease. However, this disease may affect younger individuals.

2. Gender

This disease is more often found in men than women. Therefore, men are more at risk of developing this disease than women.

3. Environment

If you are in an environment that is frequently exposed to radiation or toxic substances such as factories, workshops, lives in a house with poor ventilation, or is at high altitudes for too long, you are at risk of developing polycythemia vera.

4. Actively smoking

Smoking can increase the risk of getting polycythemia vera, due to the possible lack of oxygen flow in the blood.

Complications

What are the complications that can be caused by polycythemia vera?

If you have polycythemia vera, excess red blood cells can lead to other health complications, such as:

1. Blood clots

Excess red blood cells in the body can make the blood thicker and obstructed blood flow, making it easier for blood to clot. As for blood clots, it can cause more serious conditions, such as stroke, heart attack, or blockage of arteries in the lungs or veins in the leg or abdominal muscles.

2. Enlarged spleen

The spleen functions to fight infection and useless material in the body, such as blood cells that have been damaged or died. An increase in the number of red blood cells forces your spleen to work harder than usual, which can cause dilation or swelling.

3. Other blood disorders

In rare cases, polycythemia vera can lead to other blood diseases. One of them is myelofibrosis, which is a condition when the bone marrow is replaced with scar tissue.

In addition, this disease can cause other blood cancers, namely acute myeloid leukemia (AML). As for this disease can worsen quickly.

4. Damage to other organs

Obstructed blood flow can cause limited oxygen to other organs. This condition can cause serious conditions, such as angina (chest pain) or heart failure.

In addition, excess red blood cells can also cause a number of other complications, such as open sores to the lining of the stomach, upper small intestine or esophagus, and inflammation of the joints (gout).

There may be complications not listed above. If you need further information, please consult your doctor.

Diagnosis

How is polycythemia vera diagnosed?

Polycythemia vera may not show any symptoms for years. Often this disease is accidentally detected when the sufferer is undergoing a blood test. However, for those who feel symptoms, the doctor will diagnose this disease based on the symptoms that arise.

In the early stages, the doctor will usually ask you what symptoms you feel, your health condition, and previous medical history and perform a physical examination. After that, the doctor will ask you to undergo a series of tests to prove it.

Some of the tests that doctors usually do to diagnose polycythemia vera are:

1. Complete blood count

Complete blood count (complete blood count / CBC) aims to determine the levels of hemoglobin and hematocrit. If your hemoglobin or hematocrit levels exceed normal limits, you may have polycythemia.

A complete blood count will also check the levels of red blood cells, white blood cells, and platelets in your blood. If the levels are imbalanced and you have too many red blood cells, you may have this disorder.

2. Other blood tests

In addition to a complete blood count, you will also need to undergo other blood tests, namely:

• Blood smear . This test can show whether you have a higher than normal number of red blood cells..
• Erythropoietin (EPO) hormone level test. This test aims to measure the levels of the EPO hormone in your blood. Low EPO levels indicate polycythemia.

3. Bone marrow biopsy or aspiration

In a bone marrow biopsy test, your doctor will take a small amount of your bone marrow using a needle. This tissue sample will then be examined under a microscope to see if your bone marrow is problematic.

In addition to tissue, a sample of bone marrow fluid may also be taken through an aspiration procedure. Just like a biopsy, this fluid will also be examined under a microscope to check for problems with your bone marrow.

Medicines & Medicines

The information provided is not a substitute for medical advice. ALWAYS consult your doctor.

How to treat polycythemia vera?

Polycythemia vera is a disease that cannot be completely cured. Treatment and treatment aims to reduce blood viscosity, reduce the risk of complications, and prevent blood clots from occurring.

If polycythemia vera is treated and treated appropriately, the sufferer will have a longer life expectancy.

The type of treatment and treatment for each patient varies, depending on what the main cause is. The time and amount of treatment taken may also vary from patient to patient.

Some types of drugs and treatment for polycythemia vera are:

1. Procedure phlebotomy

This procedure is done by reducing the blood in the body, in the hope that it can reduce the levels of red blood cells. The goal is to thin the blood, so that blood flow is smoother and the risk of blood clots is reduced.

2. Take drugs

Medicines are also often given to people with polycythemia vera. These drugs include:

• Low-dose aspirin. This medication is used to reduce the risk of blood clots and pain in the legs or hands.
• Medicines to lower blood cell levels, such as hydroxyurea or interferon.
• Drugs to destroy cancer cells, such as ruxolitinib (Jakafi), especially when the patient does not respond well to hydroxyurea drugs.
• Therapy to reduce itching, like a drug selective serotonin reuptake inhibitors (SSRIs).

3. Radiation therapy

Radiation therapy is also sometimes given to patients with polycythemia vera, to help suppress overactive bone marrow cells. This can help lower the blood cell count and keep blood flow normal.

However, this treatment can increase the risk of leukemia and other blood diseases. Always consult your doctor for the right type of treatment.

Home remedies

What are some lifestyle changes or home remedies that can be done to treat polycythemia vera?

Here are the lifestyle and home remedies that can help you deal with polycythemia vera:

• Moderate intensity exercise, such as walking, to improve blood circulation and prevent blood clots.
• Avoid tobacco because it can increase the risk of heart attack or stroke due to blood clots.
• Bathing in cold water can reduce itching. Don't scratch the skin and use a moisturizer to keep the skin healthy.
• Avoid extreme temperatures to prevent poor blood circulation. Dress according to the weather in which you live.
• Be careful with wounds on hands and feet.

Prevention

How can you prevent polycythemia vera?

Polycythemia vera is a disease that cannot be prevented. However, you can reduce the risk of this disease by avoiding various risk factors that can still be changed, such as:

• Avoid exposure to radiation or chemicals that harm the body.
• Ventilate the house properly or open windows frequently to allow air to enter and reduce exposure to chemicals contained in household products.
• Avoiding things that can reduce oxygen levels in the body for a long time, such as climbing mountains, living at high altitudes, or smoking.
• Controlling lung, heart, or other diseases, which can increase the risk of this disease.
• Implement a healthy lifestyle.

If you have any questions, consult your doctor for the best solution to your problem.