Treatment
Untreated, polycythemia vera can be fatal. Research has found that the "1.5-3 years of median survival in the absence of therapy has been extended to at least 10-20 years because of new therapeutic tools."
As the condition cannot be cured, treatment focuses on treating symptoms and reducing thrombotic complications by reducing the erythrocyte levels.
Bloodletting (called venesection or phlebotomy) is one form of treatment, which often may be combined with other therapies. The removal of blood from the body reduces the blood volume and brings down the hematocrit levels; in patients with polycythemia vera, this reduces the risk of blood clots. Venesection is typically performed in people with polycythemia vera to bring their hematocrit (red blood cell percentage) down below 45 for men or 42 for women. It has been observed that phlebotomy also improves cognitive impairment.
Low dose aspirin (75–81 mg daily) is often prescribed. Research has shown that aspirin reduces the risk for various thrombotic complications.
Chemotherapy for polycythemia may be used, either for maintenance, or when the rate of bloodlettings required to maintain normal hematocrit is not acceptable, or when there is significant thrombocytosis or intractable pruritus. This is usually with a "cytoreductive agent" (hydroxyurea, also known as hydroxycarbamide).
The tendency of some practitioners to avoid chemotherapy if possible, especially in young patients, is a result of research indicating possible increased risk of transformation to acute myelogenous leukemia (AML). While hydroxyurea is considered safer in this aspect, there is still some debate about its long-term safety.
In the past, injection of radioactive isotopes (principally phosphorus-32) was used as another means to suppress the bone marrow. Such treatment is now avoided due to a high rate of AML transformation.
Other therapies include interferon injections, and in cases where secondary thrombocytosis (high platelet count) is present, anagrelide may be prescribed.
Bone marrow transplants are rarely undertaken in polycythemia patients; since this condition is non-fatal if treated and monitored, the benefits rarely outweigh the risks involved in such a procedure.
There are indications that with certain genetic markers, Erlotinib may be an additional treatment option for this condition.
Selective JAK2 inhibitors are being investigated in vitro and in clinical trials.
Read more about this topic: Polycythemia Vera
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