Polycythemia vera symptoms appear gradually, as the disease takes years to develop. This condition leads to an abnormal increase in red blood cells due to overproduction by the bone marrow. The following article will serve as a short guide to those seeking information related to polycythemia vera causes, symptoms and treatment.
There are many bone marrow disorders that cause changes in the normal blood cell count. One such myeloproliferative disorders of the bone marrow is polycythemia vera (PV). One usually sees an increase in the white blood cells leading to leukemia. However, polycythemia vera is a rare blood disorder that takes years to develop. It causes an increase in red blood cells, and in some cases increase in production of platelets as well as WBCs. However, RBCs are the ones that are produced excessively in majority of the cases of polycythemia vera. This leads to abnormal thickening of the blood due to high number of RBCs.
Causes
There are two forms of polycythemia vera that may affect a patient. The first one is a rare growth disorder of the bone marrow called primary polycythemia vera. The second one is secondary polycythemia that affects people living at high altitudes. The causes of primary polycythemia vera is gene mutation. It is thought the protein JAK2 V617F mutation causes the cells to grow continuously. Secondary polycythemia occurs when the body starts producing more red blood cells due to presence of an underlying kidney disease or lung disease. Those living at high altitudes suffer from lack of oxygen and thus, their bodies start producing more RBCs to make up for the lack of oxygen in the body. This rare disease affects people who are over the age of 40.
Symptoms
The signs and symptoms of polycythemia vera are not observed for a very long time. The polycythemia vera symptoms appear only after the blood has thickened considerably. As the blood flow reduces due to the thickened viscosity, as a result many parts of the body become oxygen starved. This reduces their working capacity and thus, many symptoms begin to appear. The symptoms that may appear in a patient include:
- Headaches
- Dizziness and weakness
- Enlarged spleen causing pressure and feelings of fullness in the left side of the abdomen
- Double vision with blind spots
- Redness on face and other parts of the body
- Itching all over the body (after a warm bath) and a burning sensation of the skin, especially over the hands and feet
- Bleeding gums
- Nosebleed
- Small cuts leading to heavy bleeding
- Unexplained weight loss
- Fatigue
- Shortness of breath
- Vertigo, tinnitus
- Easy bruising
- Thrombosis
- Budd-Chiari syndrome (in some patients)
Complications
Due to the thickening of blood, a patient may develop many complications which include the formation of blood clots in the arteries. This reduces the blood flow and increases the risk of pulmonary embolism, stroke, as well as heart attack. The spleen helps in filtering the dead and old red blood cells from the body. However, increase in number of blood cells causes the spleen to enlarge, leading to splenomegaly. The skin becomes very sensitive and one feels itchy, burning or tingling sensation on the arms, legs, hands, feet, palms and soles. Increase in blood cells causes the patient to develop peptic ulcers, gout and open sores in the stomach.
Diagnosis
Polycythemia vera is diagnosed after carrying out a complete blood test. The blood test helps to determine the number of RBCs, WBCs and platelets. It helps in detecting a high red blood cell count, elevated hemoatocrit levels, elevated hemoglobin levels and very low levels of hormone erythropoietin.
Treatment
The treatment for polycythemia vera includes phlebotomy. This means, some amount of blood is removed from the veins to help decrease the number of blood cells and blood volume. The decision to carry out phlebotomy depends on the severity of the disease. If phlebotomy is not an option, or does not help a patient, then medications are used. These medications include hydroxyurea and anagrelide that help in suppressing the bone marrow. Thus, leading to reduction in the production of blood cells. Some patients are advised to take a low dose aspirin to reduce the burning sensation on the feet, and prevent the formation of blood clots. Chemotherapy may be advised to kill cancerous cells in the body.
One needs to visit a hematologist for management of polycythemia vera symptoms. Without treatment, this condition can turn into a life-threatening situation. This is a chronic condition and one requires long-term treatment to keep this condition under control.
