Management of hydroxyurea resistant or intolerant polycythemia vera

Polycythemia vera is a Philadelphia negative myeloproliferative neoplasm characterized by erythrocytosis in which the major cause of morbidity and mortality is thrombosis. Aspirin and hematocrit reduction by venesection or cytoreductive therapy are at the cornerstone of management. First line cytoreductive therapy in high-risk patients is hydroxyurea; however, its use is associated with toxicities and resistance in a significant proportion of patients. In a disease with a long overall survival with appropriate treatment, it is imperative that other treatment options do not accelerate the risk of progression to acute leukemia. The following review will appraise the evidence of interferon, ruxolitinib, and other agents in management of hydroxyurea resistant or intolerant polycythemia vera.

Automated summary

Polycythemia vera is a myeloproliferative neoplasm characterized by erythrocytosis and thrombosis, with aspirin, venesection, or cytoreductive therapy being the main management strategies. Hydroxyurea is the first-line cytoreductive therapy for high-risk patients, but it can be associated with toxicities and resistance. Other treatment options, such as interferon and ruxolitinib, are being evaluated for hydroxyurea resistant or intolerant patients with Polycythemia vera.