Polycythemia Vera (pol-e-sy-THEE-me-uh VEER-uh) is a blood cancer disease that slowly grows to become a severe and fatal ailment if not well managed (Melo & Goldman, 2007) . The disease leads to the making of too many red blood cells whereby the excess cells thicken the blood thus slowing its flow in the body systems. At the same time, the excess red blood cells also cause complications such as clotting of the blood which may trigger a stroke or heart attack (Wasserman et al., 200 9 ) . Polycythemia Vera is however not a common disease, but a serious one after one is found to be a victim. The disease slowly develops in the body, and one may have it for many years without any knowledge. In most cases, this condition is detected during a blood testing exercise for another issue (Melo & Goldman, 2007) . Apparently, without going through treatment, Polycythemia Vera is life threating, but if one gets proper healthcare services, the signs of this disease can be eased as well as the complications and symptoms related to the disease. If symptoms of Polycythemia Vera are left unchecked for several years, the disease may progress to more serious blood cancers such as acute leukemia and myelofibrosis.
Notably, Polycythemia Vera can affect any person at any age though this disease is more prevalent for those who are sixty years and above (Melo & Goldman, 2007) . Evidently, different complications are associated with Polycythemia Vera. One of these complications is blood clotting. The increased thickness of the blood, as well as the decrease in its flow in the body, including the abnormalities in the blood platelets, increase one’s risk of blood clotting (Wasserman et al., 2009) . Consequently, blood clots can lead to heart attack, stroke, blockage of the artery in one’s lungs, which is called pulmonary embolism or in one’s veins deep in the muscles, commonly known as the deep vein thrombosis. At the same time, enlarged spleen, the splenomegaly, is also another complication of Polycythemia Vera (Melo & Goldman, 2007) . Notably, one’s spleen plays a critical role in helping in the fight against infections as well as filtering the unwanted materials such as damaged or old blood cells. In this way, the increased rate of red blood cells due to this condition makes one’s spleen to work harder than normal, thus causing the spleen to enlarge.
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Besides the aforementioned complications, Polycythemia Vera also leads to other problems that are associated with high levels of red blood cells (Melo & Goldman, 2007) . For example, such elevated levels of blood cells trigger the emergence of open sores on the inside lining of the stomach, esophagus (peptic ulcers) or upper small intestine as well as the inflammation in the body joints commonly known as gout. In some rare cases, Polycythemia Vera may lead to related blood diseases which include progressive disorders whereby the scar tissue replaces the bone marrow (myelofibrosis) as well as a myelodysplastic syndrome in which the stem cells of the body may not function or mature properly (Wasserman et al., 2009) . Finally, another blood disorder due to Polycythemia Vera is a cancer of the bone marrow or blood commonly referred to as leukemia.
Etiologies of Polycythemia Vera
Apparently, there are two types of Polycythemia which include the primary Polycythemia which is also known as the Polycythemia Vera and the Secondary Polycythemia (Melo & Goldman, 2007). Polycythemia Vera (PV) is brought about by the change or mutation in the JAK2 gene of the body. The JAK2 gene refers to a protein that helps the body in the production of blood cells. However, it is not yet known what is responsible for the changes in the JAK2 gene. On this end, it must be emphasized that PV is not an inherited disease. Therefore, it cannot be passed from the parents to their children through the body genes. In some cases, however, some families may show the tendency of JAK2 gene mutation, but the direct connection of passing such genes to children from their parents has not been established (Melo & Goldman, 2007). Critically to note is that there might as well be other genetic related factors that may play a part in causing the disease.
On the other hand, the secondary Polycythemia is not associated with JAK2 gene. According to studies, secondary Polycythemia is commonly caused by long-term exposure to low levels of oxygen (Melo & Goldman, 2007). Apparently, if individuals are exposed to prolonged periods of limited oxygen levels, their bodies may start developing and making more of the hormone erythropoietin (EPO). Connectedly, the high EPO levels may trigger one’s body to produce more red blood cells than normal thus leading to thicker blood as is the case with Polycythemia Vera (PV) (Wasserman et al., 2009). At the same time, research studies have found that lung or severe heart disease can as well cause secondary Polycythemia thus making those with such diseases is at a higher risk (Melo & Goldman, 2007). Further, those people who smoke, spend most of their times at high altitudes or are highly exposed to high carbon monoxide levels such as at workplaces or homes are also at risk of developing secondary Polycythemia.
Signs And Symptoms of Polycythemia Vera
As noted above, many of those with Polycythemia Vera may not show any symptoms or signs. However, one of the common symptoms of Polycythemia Vera includes Itchiness, which is usually experienced after one takes a warm shower or bath. At the same, most victims may have mild to strong headaches. Further, people suffering from polycythemia Vera may feel fatigued or feelings of dizziness and may experience excessive sweating. Minor bruising and bleeding are also part of signs of polycythemia Vera. The general body weakness and blurred vision coupled with shortness of breath are also symptoms of Polycythemia Vera. As mentioned earlier, Polycythemia Vera can easily lead to gout (Melo & Goldman, 2007). Therefore, a victim of Polycythemia Vera may start experiencing some painful swelling in one joint which is usually on the big toe. In addition, one may feel some numbness tingling and burning in the hands, arms, feet, and legs which are mostly accompanied by fevers. The enlarged spleen due to Polycythemia Vera can as well make one feel some bloating and fullness of the left upper abdomen. In most cases, especially when this disease has advanced, people experience unexplained weight loss.
Diagnostic Procedures of Polycythemia Vera
Based on this signs and symptoms of Polycythemia Vera, it is advisable for one to see a doctor for further check-ups. The step in diagnosis is that the doctor will take the medical history as well as perform a physical exam (Melo & Goldman, 2007). A blood test is the next step whereby if one has the Polycythemia Vera the test will show an increased number of red blood cells and in some cases the increase in levels of white blood cells and platelets. Further, the blood test will reveal a high percentage of red blood cells that account for the total volume of the blood usually called the hematocrit measurement. Other revelations from the blood test include elevated levels of protein with a high content of iron in the red blood cells as well as low levels of a hormone that helps in stimulating bone marrow for the production of new red blood cells.
Another test is the bone marrow biopsy if the doctor could be suspecting that one has Polycythemia Vera. The intention of this biopsy is to collect the bone marrow sample for further study. Finally, a gene mutation that leads to Polycythemia Vera is also tested based on the results of bone marrow biopsy.
Treatment of Polycythemia Vera
Unfortunately, as a chronic ailment, Polycythemia Vera cannot be cured (Melo & Goldman, 2007). The treatment process is focused on the reduction of the number of blood cells in the body. In most cases, the treatment process is able to reduce the risk associated with complications from these diseases thus easing the signs and symptoms. The treatment involves first taking of blood out one’s veins. The procedure of drawing blood from the veins is called phlebotomy which is usually the first option for those with Polycythemia Vera. The objective is to ensure that the number of blood cells in the body is reduced and that the volume of the blood is also reduced significantly making it easier for the blood to flow properly. The frequency of this procedure depends on how severe the condition is (Wasserman et al., 2009). Further, the doctor may decide to prescribe the Low-dose aspirin with the aim of reducing the risk of blood clots.
In addition, medication may be recommended for the reduction of blood cells (Melo & Goldman, 2007) . Medications such as hydroxyurea (Droxia, Hydrea) are used to suppress the ability of bone marrow in the production of blood cells especially for those who may not be helped by phlebotomy alone. Other types of medication include Ruxolitinib (Jakafi) for the destruction of cancer cells for those with Polycythemia Vera who cannot respond to hydroxyurea (Wasserman et al., 2009). Finally, therapy is also recommended for the reduction of itching which may involve antihistamines or ultraviolet light treatment to relieve the discomfort.
Pharmacological Considerations of Polycythemia Vera
The objective of medication or therapy for the Polycythemia Vera is the regulation and control of the myeloproliferative activities of this ailment (Melo & Goldman, 2007) . The evidence of increased white blood cells levels as well those of platelets including organomegaly show the presence of uncontrolled myeloproliferative activities which need a myelosuppressive agent. Studies that were conducted by the Polycythemia Vera Study Group (PVSG) contributed to the abandonment of long-term therapy that involved phosphorus-32 ( 32 P) and some of the conventional alkylating agents such as chlorambucil and busulfan (Melo & Goldman, 2007) . Instead, the study recommends the use of hydroxyurea (HU). However, other studies seem to show that there is possible late and a slight increase in acute leukemia cases in Polycythemia Vera patients who may have been treated with HU for more than fifteen years. In the United States, some of the approved medications include Ruxolitinib and myelofibrosis.
Prognosis of Polycythemia Vera
Although currently there is no known cure for Polycythemia Vera , there have been significant developments and effective treatments as noted above that are instrumental in reducing the symptoms of Polycythemia Vera , decreasing the reoccurrence, as well as offering many individuals suffering from Polycythemia Vera the ability to lead more satisfying and productive lives.
The median survival of untreated symptomatic for those patients with Polycythemia Vera was previously being estimated at six and eighteen months from the day of diagnosis (Melo & Goldman, 2007). However, the current survival rate of treated patients is about thirteen or more years. Despite the presence of treatment for Polycythemia Vera, studies have found that the overall mortality is much higher that of sex- or age-matched healthy population.
Prevention of Polycythemia Vera
Based on different studies on polycythemia , it has been found that it is not possible to prevent the Primary polycythemia (Polycythemia Vera) (Melo & Goldman, 2007). However, if people get proper treatment, they can delay or prevent the complications as well as the symptoms.
On the other hand, one can prevent secondary Polycythemia by avoiding scenarios that may deprive one’s body of oxygen for long periods (Melo & Goldman, 2007). In this way, one can avoid for instance mountain climbing, smoking, and living at high altitudes. At the same time, for those who may be having severe lung and heart diseases, they may undergo treatment for such diseases to improve the secondary polycythemia (Wasserman et al., 2009). It is advisable to lead a healthy lifestyle to lower the risk of lung and heart diseases which may consequently prevent secondary polycythemia.
References
Melo, J. V., & Goldman, J. M. (2007). Hematologic malignancies: Myeloproliferative disorders .
Berlin: Springer.
Wasserman, L. R., Berk, P. D., & Berlin, N. I. (200 9 ). Polycythemia vera and the
myeloproliferative disorders . Philadelphia: W.B. Saunders.
