The aim of PV treatment is to control complications and reduce the number of red cells and platelets in the blood. Venesection and/or drug therapy can be used to reduce the number of red cells and slow their production. The choice of treatment is individually tailored based on age, tolerance of venesection, red blood cell count, and history of clotting or bleeding complications. The drugs that we use to reduce red cell numbers include:

Hydroxycarbamide: This drug interferes with cell metabolism and can reduce red blood cell and platelet counts. It is a daily treatment, given in capsule form. Patients taking this drug will need their blood counts monitored every two to three months. Side effects include some darkening of skin, mouth and leg ulcers, and sometimes stomach problems. Hydroxyurea can damage cells and may affect fertility. Women who are pregnant or those trying to conceive should not tale hydroxycarbamide. There is a small chance that this drug may increase the risk of acute leukaemia after 10-15 years of treatment.

Melphalan, busulphan and phosphorous: Melphalan, busulphan or radioactive phosphorous were the main therapies used to treat PV in the past. However, these drugs can damage bone marrow and make patients infertile. These treatments also increase the risk of acute leukaemia. Haematologists still use busulphan and phosphorous to treat PV in certain cases: when their are side effects from other drugs, when these drugs are not working, or when it is difficult to take the hydroxycarbamide tablets.

Interferon Alpha: This is a natural agent that reduces the production of bone marrow cells. It is used in some PV patients wanting to preserve fertility and can be given to pregnant women. It is given by injection three times a week. The side effects are mainly flu-like symptoms, hair loss, depression, liver and thyroid changes. Evidence suggests that this drug probably does not increase the risk of leukaemia.

Anagrelide: Anagrelide is a drug that lowers the red blood cell count by reducing the size of the red blood cell-producing cells. It also should not affect fertility or increase the risk of developing acute leukaemia. Anagrelide can have side effects including headache, palpitations and fluid retention. It is not suitable for pregnancy. It is used with caution in heart disease.

In Europe, anagrelide is only licensed as a second line treatment for polycythaemia vera. The PT.1 trial suggests it may not be as good as hydroxycarbamide in preventing clotting and bleeding complications. It also seems that anagrelide is not as good as hydroxycarbamide at reducing transformation to myelofibrosis.

Other treatments:

In addition to using drugs or venesection, we treat a number of patients with aspirin to reduce the likelihood of complications. Aspirin may increase bleeding in patients with PV especially from the gut, although this is unlikely if the blood count is well controlled and the patient has no pre-existing ulcer or bleeding history.

We control gout by reducing the number of red cells and using allopurinol to reduce the symptoms. We treat an acute attack of gout with painkillers. Itching can be a particularly difficult problem to control. We can use creams and ranitidine to ease the symptoms. To read more about controlling itching, please click here:
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