Precise regulating mechanism in the body normalizes the production of red blood cells in the bone marrow, so that the number of red cells produced is equal to the number of cells that are decaying. In polycythemia, this mechanism is disrupted, and bone marrow produces too many red blood cells, while the number of cells dying is permanent. The consequence is an increased number of red cells in the blood.
The disorder occurs in three main forms. Primary polycythemia (or polytemias rubra vera), overexpression of red cells, is the most difficult form of this disease.
Secondary polytemias appears as a symptom of another, basic disease, e.g. severe chronic bronchitis. Because of this basic red blood cell disease, they can not get enough oxygen to shed it on the body’s tissues, and hence the bone marrow will react with more red cell production. The outcome depends on the success in treating the underlying disease.
The third form of this disorder is pseudo-polycythemia (relative polytemias) which, as the name suggests, is not a real polycythemia because her cause is not a problem in the bone marrow. Instead, large red cell density is a consequence of a blood plasma deficiency, a liquid in which the blood cells are scattered. The cause of blood plasma deficiency is not known, but there is an association of this disease with obesity, stress and excessive alcohol consumption. Although pseudo-polycythemia itself is not dangerous to health and rarely requires treatment, the aforementioned lifestyle associated with this disease can lead to other disorders. Therefore, it is recommended that patients reduce the amount of food and drink.
In the rest of this article we will discuss primary polycythemia, the most difficult form of the disease. Produced red blood cells are healthy, but are so numerous and so concentrated that they have a harmful effect on the body.
Typical symptoms of primary polycythemia are frequent headaches, dizziness, feeling of “fullness”, and blurred vision. Itching can also occur – often strong and enhanced after a hot bath. During the medical examination. a doctor may find another symptom – enlargement of the spleen, a swelling just below the ribs on the left side of the abdomen.
Primary polycythemia is very rare; only one person per 50 000 suffers from it annually. Disease affects both sexes equally and occurs mostly in people over 40 years of age.
Although primary polycythemia is incurable, this disease needs many years to develop, and since the begining of the treatment many patients survive for up to 20 years. Abnormal red cell concentrations can lead to coronary thrombosis, deep vein thrombosis, stroke, gout or kidney damage.
What to do?
Anyone with the symptoms of polycythemia must contact a doctor who will refer him/her to blood tests. If those tests shows that patinent has polycythemia, a special blood test (called blood volume analysis) will be performed to determine the type of polycythemia. If the test is positive, your doctor will refer you to other tests, such as x-rays (pediatric intravenous).
In most cases of primary polycythemia, treatment is performed outpatient. Treatment is primarily directed at reducing the number of red blood cells in order to eliminate the risk of thrombosis. To achieve this, half a liter of blood is taken from the patient (from the vein on his hand) to remove excessive blood cells. In mild cases, the only treatment needed will be blood extraction every three to four months. With drugs, such as radioactive phosphorus (which is injected into the vein) or cytostatic tablets, the excessive production of red cells is usually successfully controlled. Treatment can be effective between three months and several years. When the symptoms reappear a new treatment is given but, eventually, the disease will stop reacting to all forms of treatment.