Polycythemia(also known as polycythemia polyglobullia) is a disease state in which the hematocrit(the volume percentage of red blood cells in the blood is increased).
Polycythemia is normally reported in terms of increased hematocrit (hematocrit is the ratio of the volume of red blood cells to the total volume of blood) or hemoglobin concentration (hemoglobin is a protein responsible for transporting oxygen in the blood).
- Hematocrit (HCT): Polycythemia is considered when the hematocrit is greater than 48% in women and 52% in men.
- Hemoglobin (HGB): Polycythemia is considered when a hemoglobin level of greater than 16.5g/dL in women or hemoglobin level greater than18.5 g/dL in men.
Polycythemia can be divided into two categories; primary and secondary.
- Primary polycythemia: In primary polycythemia the increase in red blood cells is due to inherent problems in the process of red blood cell production.
- Secondary polycythemia: Secondary polycythemia generally occurs as a response to other factors or underlying conditions that promote red blood cell production.
Red cell production (erythropoiesis) takes place in the bone marrow through a complex sequence of tightly regulated steps. The main regulator of the red cell production is the hormone erythropoietin (EPO). This hormone is largely secreted by the kidneys, although, about 10% may be produced and secreted by the liver.
Symptoms:
The symptpms of polycythemia are as follows,
- Headaches
- Double vision or seeing dark or blind spots that come and go
- Itching all over your body, especially after you’ve been in warm or hot water
- Sweating, especially at night
- Reddened face that looks like sunburn or blushing
- Weakness
- Dizziness
- Weight loss
- Shortness of breath
- Tingling or burning in your hands or feet
- Painful swelling of a joint
Diagnosis:
It is done by CBC.CBC measures the following factors in your blood:
- the number of red blood cells
- the number of white blood cells
- the number of platelets
- the amount of hemoglobin (a protein that carries oxygen)
- the percentage of space taken up by red blood cells in the blood, known as the hematocrit
PV is a chronic condition that doesn’t have a cure. However, treatment can help you manage its symptoms and help prevent complications. Your doctor will prescribe a treatment plan based on your risk of developing blood clots.
Treatment for low-risk people
Typical treatment for those at low risk of blood clots includes two things: aspirin and a procedure called phlebotomy.
- Low-dose aspirin. Aspirin affects the platelets in your blood, decreasing your risk of forming blood clots.
- Phlebotomy. Using a needle, your doctor will remove a small amount of blood from one of your veins. This helps reduce your red blood cell count. You’ll typically have this treatment about once a week, and then once every few months until your hematocrit level is closer to normal.
Treatment for high-risk people
In addition to aspirin and phlebotomy, people at high risk of blood clots may require more specialized treatment, such as other medications. These can include:
- Interferon alpha. This drug helps your immune system fight off the overactive bone marrow cells that are part of PV. It can also block your body from making too many red blood cells. Like hydroxyurea, interferon alpha is used off-label to treat PV.
- Hydroxyurea (Droxia, Hydrea). This is a cancer drug that prevents your body from making too many red blood cells. It reduces your risk of blood clots. Hydroxyurea is used to treat PV.
- Busulfan (Myleran). This cancer drug is approved to treat leukemia, but it can be used off-label to treat PV.
- Ruxolitinib (Jakafi). This is the only drug approved by the U.S. Food and Drug Administration to treat PV. Your doctor may prescribe this drug if you can’t tolerate hydroxyurea, or if hydroxyurea doesn’t lower your blood count enough. Ruxolitinib works by inhibiting growth factors responsible for creating red blood cells and immune system functioning.
