Overview of Polycythemia Vera: Choosing a Treatment That’s Right for You

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Topics include: Treatments and Understanding

Although polycythemia vera (PV) may develop slowly and show now symptoms for years, it’s essential that manage and treat the condition. Tune in to hear Dr. David Snyder, Associate Chair of Hematology and Hematopoietic Cell Transplantation at City of Hope, as he explains PV on a cellular and hormonal level, mutations associated, and options for treatment.

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Transcript

Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you. 

Beth Kart Probert:

Could you give our viewers a little background on PV? And then we’ll talk a little further about how the mutation plays a role with that and what we look for in that.

Dr. Snyder:           

PV is polycythemia vera. It’s an overproduction of red blood cells. It shares the properties with its cousins, ET and myelofibrosis, the JAK-STAT pathway is overactivated, usually because of the JAK2V617F mutation. So the cells in the bone marrow produce red blood cells become autonomous, if you will. They’re always turned on. 

Normally the body regulates very well how many red cells are produced by the bone marrow. If the tissues in the body sense that there’s not enough oxygen coming and the message gets sent through a hormone called erythropoietin goes back to the bone marrow, stimulates more red cell production. More red cells bring more hemoglobin, brings more oxygen to the tissues, and then erythropoietin production is turned off. 

In P. vera, that production of red blood cells becomes independent of the erythropoietin signal, so those precursors are always churning out red blood cells, even though the body doesn’t need them. So the hemoglobin hematocrit can go very high, and that gets people into problems with risk of thrombosis as the main issue. Also, there are systemic symptoms that we talked about in myelofibrosis can also be seen in polycythemia vera.

So the treatment goals there are to again control the risk of thrombosis by keeping the hematocrit at a safe level, and there have been some well-designed trials now showing that keeping the hematocrit under 45 percent is the desired goal. Some hematologists would even be a little more blasé about it and say well, it’s under 50 percent. That’s okay. But there are now clear data to show that you’re doing a disservice to your patient by allowing hematocrits to get over 45 percent. And frankly for women, it may even be better to shoot for 42 percent as the target.

And so the question is how do you get there, and phlebotomy is certainly the most direct mechanical way, if you will, of doing that. But cytoreduction with interferon is one option. Hydroxyurea (Hydrea) is probably the most common drug used in that setting.

And now for the last few years, ruxolitinib (Jakafi) is also FDA approved as a treatment for patients with polycythemia vera who have become intolerant to hydroxyurea or resistant 

Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you. 

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Page last updated on October 13, 2017