Overview
Polycythemia vera is a rare type of blood cancer characterized by an overproduction of red blood cells in the bone marrow. This causes the blood to become thick and slow, which can result in serious issues like blood clots. The condition usually progresses slowly, and people may not show noticeable symptoms. However, some individuals might experience headaches, dizziness, fatigue, blurred vision, itchiness, and unusual bleeding. If left untreated, polycythemia vera can be life-threatening, but proper medical care can help manage symptoms and prevent complications. The cause of the gene mutation responsible for polycythemia vera is unknown, and it typically does not run in families.
Understanding the disease
Symptoms
Symptoms of polycythemia vera may include headache, dizziness, fatigue, blurred vision, itchiness, especially after a warm bath or shower, unusual bleeding, such as a nosebleed or bleeding gums, painful swelling of one joint, often the big toe, shortness of breath and difficulty breathing when lying down.
Risk Factors
The risk factors for developing polycythemia vera include:
- Age: Polycythemia vera can occur at any age, but it's more common in adults between 50 and 75 years old.
- Gender: Men are more likely to get polycythemia vera, but women tend to get the disease at younger ages.
- Gene mutation: Polycythemia vera occurs when a mutation in a gene causes a problem with blood cell production. The cause of the gene mutation is unknown, but it's generally not inherited from your parents.
Development
Polycythemia vera is a rare type of blood cancer that occurs when there is a mutation in a gene that causes a problem with blood cell production. The bone marrow produces too many red blood cells, white blood cells, and platelets, leading to thickened blood that can cause serious problems such as blood clots. The cause of the gene mutation is unknown, and it is generally not inherited from parents. The condition can develop at any age, but it is more common in adults between 50 and 75, with men being more likely to develop the disease and women getting it at younger ages. There is no cure for polycythemia vera, but proper medical care can help manage the symptoms and complications.
Assessment and Diagnosis
Triage
You should see a doctor if you have signs or symptoms of polycythemia vera, which may include headache, dizziness, fatigue, blurred vision, itchiness, unusual bleeding, painful swelling of one joint, shortness of breath, and difficulty breathing when lying down. These symptoms may be vague or more specific, and it is important to consult your doctor for proper medical evaluation and care.
Diagnosis
To diagnose polycythemia vera, doctors will take a detailed medical history, perform a physical exam, and conduct blood tests. The blood tests may reveal more red blood cells than normal, an increased hematocrit measurement, and elevated hemoglobin levels. Your doctor may also recommend further tests like imaging tests (such as chest X-rays, CT, or MRI) to determine the extent of the disease.
Management and Treatment
Complications
Polycythemia vera can lead to several potential complications, including:
- Blood clots: The thickened blood caused by excess red blood cells can lead to blood clots, which can be life-threatening if they block blood flow to vital organs like the heart, brain, or lungs.
- Heart problems: Polycythemia vera can cause inflammation of the heart muscle (myocarditis), which may lead to chest pain, shortness of breath, and heart failure.
- Stroke: The thickened blood can also increase the risk of blood clots traveling to the brain, causing a stroke.
- Anemia: Although polycythemia vera involves an excess of red blood cells, some people may also develop anemia due to a decrease in other blood cell types, such as white blood cells or platelets.
- Myelofibrosis: In rare cases, polycythemia vera can progress to myelofibrosis, a disorder in which the bone marrow is replaced with scar tissue, leading to decreased production of blood cells.
- Acute leukemia: In some cases, polycythemia vera can progress to acute leukemia, a cancer of the blood and bone marrow.
- Infections: People with polycythemia vera may be more susceptible to infections due to a decreased number of white blood cells.
- Liver problems: Polycythemia vera can cause mild liver inflammation (hepatitis) and occasionally lead to jaundice (yellowing of the skin and eyes). It is important to seek medical attention if you experience any signs or symptoms of polycythemia vera, as proper medical care can help manage the condition and reduce the risk of complications.
Treatment
To treat polycythemia vera, the primary approach is to manage symptoms and reduce complications. The following treatments may be recommended:
- Frequent blood withdrawals (phlebotomy): This is the most common treatment for polycythemia vera. It involves removing excess blood cells through a needle in a vein, similar to donating blood. The frequency of these blood withdrawals depends on the severity of the condition.
- Medications to reduce red blood cell count: If phlebotomy alone doesn't provide sufficient relief, medications may be prescribed to reduce the number of red blood cells in your bloodstream. Examples include hydroxyurea, interferon alfa-2b, ruxolitinib, busulfan, and others.
- Heart medications: Your doctor will likely prescribe medications to control risk factors for heart and blood vessel diseases, such as high blood pressure, diabetes, and abnormal cholesterol levels. Low-dose aspirin may also be recommended to reduce the risk of blood clots and alleviate pain in your feet or hands.
- Self-care: Engaging in moderate exercise, avoiding tobacco, and following a healthy diet can help improve your overall health and well-being. It's important to work closely with your doctor to develop a personalized treatment plan based on your individual needs and medical history. Regular check-ups and monitoring will help ensure the effectiveness of your treatment and the management of any potential complications.