Risk Assessment and Treatment of Polycythemia Vera


Yelena Ginzburg, MD, discusses the prognosis and standard-of-care treatment in patients diagnosed with polycythemia vera.

Yelena Ginzburg, MD, associate professor of medicine, hematology, and medical oncology at Mount Sinai, discusses the prognosis and standard-of-care treatment in patients diagnosed with polycythemia vera.

Polycythemia vera is one of several types of myeloproliferative neoplasms. It is often associated with long-term survival up to 20 years. Lower-risk patients tend to be younger and have less risk of thromboembolic events, which have a high mortality risk, while high-risk patients tend to be older and may have a history of thromboembolic events.

Therapeutic phlebotomy is often the primary treatment for polycythemia vera, especially for patients with low-risk disease. Higher-risk patients may be treated with cytoreductive agents such as hydroxyurea and interferon as first-line therapy.

The Janus kinase inhibitor ruxolitinib (Jakafi) may be used as second-line therapy based on results from the RESPONSE trial (NCT01243944) that showed efficacy in patients who failed to respond to hydroxyurea. The primary end point of hemocrit control at 32 weeks was achieved in 21% of patients receiving ruxolitinib versus only 1% of those receiving standard therapy, as well as greater overall hemocrit control and more patients with spleen reduction.

Even with cytoreductive therapy, high levels of therapeutic phlebotomy may be required, which increases the risk of thrombosis in these patients, Ginzburg says.


0:08 | Polycythemia vera is a chronic myeloproliferative disease, one of several. It is associated often, given its chronicity, with a long period of survival, including sometimes up to 20 years post diagnosis. There are groups of patients both better categorized into low risk versus high risk. The low-risk patients are younger and do not have a history of thromboembolic events. The higher-risk population is older and/or has a history of a thromboembolic event.

Patients are often treated with therapeutic phlebotomy, that's the mainstay of treatment, especially in the low-risk population. In the high-risk population, patients are often treated with cytoreductive agents of which hydroxyurea and interferon are both first line and ruxolitinib [Jakafi] is considered second line. So, despite cytoreductive therapy, some patients still require high levels of therapeutic phlebotomy and there is some risk of thrombosis that's higher in patients who require a higher rate of therapeutic phlebotomy.

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