This is a type of blood cancer that occurs in the bone marrow, where new blood cells are produced. It’s a rare neoplasm of the bone marrow, and mainly affects the production of erythrocytes, also known as red blood cells. This condition can also affect the production of other cells in the blood, such as the platelets, and white blood cells. This disease leads to problems because it causes the blood to become thicker, due to the abnormal increase in the number of red blood cells. Some of the signs and symptoms of this include pruritus, severe pain in the extremities, such as in the legs and hands. They might also present with discoloration of the skin. Other symptoms presented by patients include gouty arthritis, peptic ulcer disease and so on. This disease doesn’t occur suddenly but might take years to develop. Patients might also not present with any signs and symptoms for a long time. Majority of polycythemia vera diagnosis are accidental. They’re mostly detected when persons affected are undergoing a blood test, for other purposes disease occurs largely due to a mutation in the genes responsible for blood cell production. It’s one of the diseases that belong to the myeloproliferative neoplasms. The body loses control of the production of blood cells, particularly red blood cells. The mutation in the protein JAK2, also known as Janus Kinase 2, is responsible for this disease. The main cause of this mutation is not known yet. The treatment of this disease depends on some factors. Some of the factors include age and the overall state of the patient. Patients that only have mild symptoms might not need treatments. All they need might just be a regular medical check-up. On the other hand, people with severe symptoms might have to undergo treatment procedures such as chemotherapy, phlebotomy, which is the drawing out of blood out of the veins, and so on. Researchers are working on ways to use shock waves in the treatment of the disease.
What Are The Signs Of Polycythaemia Vera?
Persons affected with this disease might not present with any symptom for a long time. Most patients start presenting with signs and symptoms in the later stage of the disease. Below are some of the common symptoms of polycythemia vera:
- Pruritus: This condition is also known as itching. It’s one of the classic symptoms of the disease. It mostly occurs when the affected person exposes himself to warm water. This does occur when having a warm bath. Studies have shown that about 40% of patients present with itching. Itching occurs due to the abnormal release of histamine, and also prostaglandin production.
- Gouty arthritis: This affects about 20% of patients. It is the condition in which the patient presents with pain and discomfort in the bones and joints. The most commonly affected joints include the knee, ankle and the bones of the extremities.
- Peptic ulcer disease: This is a condition in which an individual develops painful sores in the lining of the abdominal tract. This occurs due to the excess production of acid in the stomach. Peptic ulcer disease includes both stomach ulcer and duodenal ulcer. In the case of polycythemia vera, it occurs due to the surge in histamine release from the mast cells. It also has a link to the ulcer-causing bacteria, Helicobacter Pylori.
- Erythromelalgia: This is also one of the classic symptoms of Polycythemia It’s a condition in which the patient feels a burning sensation in the extremities, such as the hands and the feet. There are other conditions that are often associated with this disease. Some of these conditions include a change in the color of the skin. Patients’ skin usually changes color to red or blue. Erythromelalgia occurs because of the increase in the number of platelet count. The increase in platelet count causes the clumping of the cells, which might eventually form small blood clots in the vessels.
- Thrombosis: Patients affected with polycythemia vera have a high risk of developing thrombosis. This is a condition in which there is the development of blood clots in the vessels. This condition might lead to severe complications if not well treated. Some of these complications include heart attack, deep venous thrombosis, stroke and so on.
- Fatigue: Patients get tired too easily, even when doing activities they use to do without stress.
- Double vision
- Nocturnal sweating: Patients tend to sweat a lot, especially at night.
- Loss of weight
- Dizziness
What Are The Causes Of Polycythemia Vera?
Polycythaemia vera is one of the diseases that belong to the myeloproliferative neoplasms. It occurs as a result of the mutation of the gene that controls blood cell production. Typically, some genes are responsible for the production of blood cells. However, the mutation in the protein JAK2 leads to an abnormality in the process. Studies have shown that the majority of those affected with the disease have a mutation of this protein. The exact cause of this mutation isn’t known yet, but it’s certain that it isn’t inherited.
What Are The Complications Of Polycythemia Vera?
Polycythemia vera has various complications. Some of them are as follows;
- Blood clots: There is the formation of blood clots due to the increased thickness of the blood, and the reduced flow in the vessels.
- Organomegaly: One of the complications of polycythemia vera is the enlargement of organs. One of these is the enlargement of the spleen. This occurs because the spleen helps defend the body against infections, and also remove waste materials.
How Is Polycythemia Vera Treated?
This disease can be fatal if left untreated. This disease cannot be treated yet. However, the signs and symptoms of the disease can be improved through proper management. The most common and one of the most effective treatment procedure is phlebotomy. This is a process in which blood is removed from the body, stimulating iron deficiency, hence reducing the hemoglobin level, and also reducing the risk of developing blood clots. Other treatment methods include chemotherapy and so on.
References
Pahl, H. (2000). Towards a molecular understanding of polycythemia rubra vera. European Journal of Biochemistry, 267(12), pp.3395-3401.
Saini, K., Patnaik, M., and Tefferi, A. (2010). Polycythemia vera-associated pruritus and its management. European Journal of Clinical Investigation, 40(9), pp.828-834.
Spivak, J. (2014). The polycythemia vera stem cell. Leukemia Supplements, 3(S1), pp.S23-S24.
