What Are the Available Treatments for PV?
Many of the treatment options for PV are designed to manage the disease by returning hematocrit levels to normal values. However, the circumstances are different for every PV patient and asymptomatic patients may not require treatment for extended periods of time. Routine monitoring by a physician is recommended for all PV patients, regardless of symptoms.
When treatment is required, common treatment options for PV include:
Phlebotomy is the removal of blood to reduce the number of blood cells. With fewer blood cells, the blood is thinner and flows more easily, improving symptoms and reducing the risk for blood clotting. This procedure is typically done to meet target blood count goals that are determined by the physician, taking into consideration the patient’s sex and other factors.
Most, if not all PV sufferers are prescribed a low-dose aspirin treatment. Since aspirin prevents platelets from sticking together, it reduces the occurrence of blood clots that can cause life-threatening heart attacks or strokes.
Combined with low-dose aspiring, the regular maintenance of a hematocrit below .45 for men and .42 for women is currently accepted as a non-leukomegenic approach and a first choice treatment for recently diagnosed, low-risk PV patients.
If phlebotomy and low-dose aspirin are not effective or appropriate, or if a patient is consider higher risk for blood clotting, physicians may prescribe medicine to lower red blood count and relieve symptoms, including:
Hydroxyurea is often prescribed for PV patients at high risk for blood clots, based on age and prior history of blood clotting.
Jakafi is the first FDA-approved treatment for PV patients who have an inadequate response to or cannot tolerate hydroxyurea. Jakafi inhibits the JAK 1 and 2 enzymes that are involved in regulating blood and immunological functioning. It also helps decrease the occurrence of an enlarged spleen (splenomegaly) and the need for phlebotomy. Patients do not need to be JAK2 positive to take Jakafi, though the great majority with PV harbor this mutation.
Some PV patients have experienced anemia with or without splenomegaly while on Jakafi. Please discuss in depth with your doctor upon making any treatment decisions.
Younger patients who require treatment and women of childbearing age are often treated with pegylated interferon because it has not been shown to cause birth defects. Since Pegasys was developed for Hepatitis C and not MPN, it is considered an “off-label” medication. There are several clinical trials currently being conducted to evaluate Pegasys in people with MPNs.
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