Although hydroxyurea is considered the first-line cytoreductive therapy in high-risk patients with polycythemia vera or essential thrombocythemia, approximately 20-25% of patients develop resistance or intolerance and they need an alternative therapy. Anagrelide is the treatment of choice in patients with essential thrombocythemia intolerant or with resistance to hydroxyurea. Anagrelide is usually well tolerated. Although there is concern about the increased risk of cardiac side effects, in most cases these are mild, and easily manageable. In this paper, the available evidence about the management of patients with myeloproliferative neoplasms, with a special focus on the side effects of drug therapies is reviewed.
Keywords: BCR/ABL-negative chronic myeloproliferative neoplasms; Philadelphia-negative chronic myeloproliferative neoplasms; anagrelide; essential thrombocythemia; hydroxyurea; interferon-α; polycythemia vera; primary myelofibrosis.