Polycythemia vera is a condition when there are too many red blood cells in the body. This disease is a type of blood cancer that abnormal cell growth and development begins in the bone marrow.
Bone marrow is responsible for producing 3 types of blood cells, namely red blood cells, white blood cells, and platelets. Under normal conditions, the body regulates the number of blood cells it produces to suit its needs.
Polycystemia vera occurs when the bone marrow produces too many red blood cells. Under normal conditions, the number of red blood cells is as follows:
- 4.7–6.1 million cells per microliter of blood in men
- 4.2–5.4 million cells per microliter of blood in women
- 4.0–5.5 million cells per microliter of blood in children
Red blood cells function to carry oxygen in the blood throughout the body. If the amount is too much, the blood will thicken and flow more slowly. This condition makes the organs of the body do not get an adequate supply of oxygen.
Causes of Polycythemia Vera
Polycystemia vera is caused by a mutation in the JAK2 gene. The JAK2 gene plays a role in controlling the production of a protein that helps the formation of blood cells. Although this mutation can occur due to heredity, but in the majority of cases, PV occurs in the absence of heredity.
Politicemia vera can affect anyone, but is more common in women and people aged 50–75 years.
Symptoms of Polycythemia Vera
Polycythemia vera often causes no symptoms, even if it persists for years. This left the person with politicemia vera unaware of his illness, until he underwent blood tests to check for other conditions.
In some patients, politicemia vera can cause dizziness, headaches, blurred vision, and fatigue. Other symptoms specific to politicemia vera may include:
- Itching, especially after a warm bath
- Reddish skin, especially on the face, hands and feet
- Pruritus (itchy skin)
- Joint pain and swelling, especially in the big toe
- Bleeding, such as nosebleeds or bleeding gums
- Stiffness, tingling, weakness, or pain in the hands or feet
- Shortness of breath, especially when lying down
- Sensation of bloating, bloating, abdominal pain, and discomfort, especially after eating, due to an enlarged spleen
When to go to the doctor
Immediately consult a doctor if the above symptoms appear, especially if the symptoms do not improve. Early examination and treatment can prevent you from serious complications, such as heart attack, stroke, pulmonary embolism, and deep vein thrombosis.
Diagnosis of Polycythemia Vera
The doctor will ask about the patient's symptoms and perform a physical examination. After that, the doctor will perform additional examinations in the form of:
Complete blood count
The patient's complete blood count will show:
- An increase in the number of red blood cells accompanied by an increase in the number of platelets and white blood cells
- Increased hematocrit, which is the percentage ratio of red blood cells to blood volume
- Increased levels of hemoglobin, which is an iron-rich protein in red blood cells
- Decreased levels of erythropoietin, a hormone that stimulates bone marrow cells to produce red blood cells
Genetic test
Genetic testing is done by taking a sample of the patient's blood. This blood sample is then examined to detect mutations in the JAK2 gene.
Bone marrow biopsy
A bone marrow biopsy can help confirm the diagnosis of politicemia vera. A bone marrow biopsy is performed by taking a sample of bone marrow fluid for examination in a laboratory.
abdominal ultrasound
Abdominal ultrasound may also be done to detect whether there is a problem with the kidneys or not.
Treatment of Polycythemia Vera
Treatment for polycythemia vera aims to relieve symptoms, reduce red blood cell counts, and prevent complications.
To relieve symptoms, your doctor will prescribe low-dose aspirin to reduce burning in your hands and feet and reduce the risk of blood clots forming. Meanwhile, to relieve itching, the doctor will prescribe antihistamine drugs.
To reduce the number of red blood cells, doctors can use the following methods:
- Remove blood through a vein using a syringe (phlebotomy)
- Prescribing drugs to suppress the bone marrow's ability to produce blood cells, such as interferon alpha-2b and hydroxyurea
- Give drugs to destroy cancer cells, such as ruxolitinib and busulfan
To help with treatment from a doctor, people with polycythemia vera can take the following simple steps:
- Do moderate-intensity exercise regularly, such as stretching your legs or walking.
- Avoid environments with low air pressure, such as mountains or highlands.
- Use clothes that can warm the body in cold weather, avoid sun exposure in hot weather, and drink lots of water.
- Take a cold shower and apply moisturizer regularly to the skin. Avoid hot baths, saunas, and scratching itchy skin.
- Check your hands and feet regularly and tell your doctor right away if you have sores on these areas.
- Stop smoking.
It should be noted that politicemia vera cannot be cured. The above treatment methods only aim to relieve symptoms and prevent complications.
Complications of Polycythemia Vera
An increase in the number of red blood cells in polycythemia vera will cause blood to thicken and form blood clots. This condition can increase the risk of stroke, heart attack, deep vein thrombosis (DVT), Budd-Chiari syndrome, or pulmonary embolism.
In addition, polycythemia vera can also increase the risk of complications and other diseases, such as:
- Arthritis
- stomach ulcer
- Spleen enlargement
- Other blood cancers, such as myelofibrosis or acute myeloid leukemia (AML)
Prevention of Polycythemia Vera
Polycystemia vera is caused by gene mutations, therefore this condition cannot be prevented. However, people with politicemia vera can have a longer life if they undergo treatment and have their condition checked regularly.
Patients with politicemia vera who undergo treatment can live up to several decades. Conversely, if left untreated, people with politicemia vera only have a life expectancy of less than 2 years.