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Polycythemia Vera also known as (Primary Polycythemia) is a rare chronic condition characterised by overproduction and abnormal increase in the blood cell counts. Elevated blood cell count makes blood thicker than usual and more prone to clots and also causes a decrease in the flow.
This makes it harder to provide the oxygen needed to the organs, which leads to serious complications in the long run. Mostly diagnosed during routine blood tests, Polycythemia Vera is an insidious condition and can be life-threatening if left untreated. It can be managed by medical intervention and symptomatic treatment to assist the patient in leading a normal healthy lifestyle.
Bone marrow is responsible for the synthesis of red blood cells, white blood cells and platelets. Overproduction of one or more types of these blood cells in the bone marrow causes Polycythemia Vera. Majority of the cases show an increase in red blood cell count but there can also be an increase in either white blood cells or platelets. This disorder is caused by JAK2 or TET2 gene mutation which affects bone marrow activity and causes abnormal proliferation of blood cells. In most of the cases the JAK2 gene is affected.
Based on the causative factor, Polycythemia can be classified as: ·
Also known as Polycythemia Vera, it is caused due to JAK2 gene mutation. JAK2 gene synthesises the protein responsible for production of blood cells, and a mutation in this gene leads to protein dysfunction leading to abnormal proliferation. Various hereditary factors play a role in the gene mutation.
Factors unrelated to gene mutation such as long-term oxygen deprivation, heart lung diseases, exposure to Carbon Mono-oxide, smoking, and certain tumours releasing EPO can also cause polycythemia.
Signs and symptoms of Polycythemia Vera include:
Polycythemia Vera is caused by genetic mutation, which can be hereditary or can happen without a familial connection, and can lead to serious complications based on the tendency to form clots. Various factors that increase the risk of blood clots are:
A detailed medical history and physical exam is required along with charting of symptoms. Polycythemia Vera is generally diagnosed over a routine blood test called the Complete Blood count that measures hematocrit ratio, glycosylated haemoglobin, number of red and white blood cells and platelets. For advanced testing a bone marrow biopsy is done to confirm. Most of the tests are routine lab tests and do not need overnight hospitalisation.
Below are the tests performed:
In a patient with Polycythemia Vera, the following can be seen: ·
Polycythemia Vera does not have a cure. The current treatment modalities are to decrease the risk of associated complications that arise with the disorder. Palliative care and symptom modulation is employed to maintain a normal lifestyle. Treatment includes managing symptoms and lifestyle changes. ·
Timely blood withdrawals will decrease the volume of blood and prevent complications related to clots and circulation. ·
To relieve the discomfort caused due to itching, medications such as Antihistamines and UV light treatment can be recommended. ·
Certain medications such as Interferon alfa, Ruxolitinib, Busulfan can be prescribed to decrease the number of red blood cells. ·
Polycythemia Vera increases complications related to the heart and predisposes to various issues. Low doses of Aspirin can help reduce platelet aggregation and reduce the burning sensation of the skin. ·
With chronic conditions such as Polycythemia Vera, lifestyle changes are instrumental in maintaining the normal functioning of the body.
If you are facing any similar signs or symptoms please contact the BLK-Max team to schedule an appointment at : +91-11-30403040