How Likely Is It That Polycythemia Vera (PV) Will Become Myelofibrosis?

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As part of our Ask the Expert series, Dr. Srdan Verstovsek of MD Anderson Cancer Center in Houston responds to a question from the MPN community about the likelihood of polycythemia vera (PV) becoming myelofibrosis (MF). Dr. Verstovsek starts by discussing how MPNs are interrelated and what researchers currently understand about disease progression.

Sponsored by The Patient Empowerment Network, which received an educational grant from Incyte Corporation.

 

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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. 

Srdan Verstovsek:

The question is how likely is for a PV to become myelofibrosis? We understand at this point in time that essential thrombocythemia, polycythemia vera and myelofibrosis is a group of diseases that are interrelated, and the continuum of one disease where the common abnormality in all these patients is the hyperactive of the signaling pathway inside the cells, the so-called JAK-STAT pathway. Yet patients present with different clinical problems that lead to different complications and different life expectancy, from ET that is considered by and large benign with an excellent life expectancy, to myelofibrosis that has a lot of complication,s and unfortunately, shortens the life expectancy of the patients.

We know that patients change from one to the other. Some patients can change to ET to PV, some patients can change from ET to myelofibrosis, and PV patients can change to myelofibrosis.  The risk is rather low, and it requires a lot of time to pass for one to change from polycythemia vera to myelofibrosis.

There are a number of different studies that retrospectively looked in different databases from Italy to Spain to England to United States to see what is at risk, and it seems to be from some of these studies to be about between 5 and 15 percent of the patients to transform from PV to myelofibrosis after having the disease about 15 to 20 years.

So this is something that may or may not happen in the future in the patients that we see at the beginning.  We don't have a real way of predicting that.  The way to follow patients with PV is to see them regularly, perhaps at the beginning more often than later, perhaps twice a year or once a year when the disease is stable just to make sure that things are under good control change and there is no signs of change.

The signs of change are seen in the blood. There is no real reason to do proactive checking of the bone marrow for signs of fibrosis.  If there is a change, it will result in a change in blood. The patients will become anemic not related to the therapy. They may have big spleen symptoms.  They may have bone marrow cells in blood, overall condition of the body deteriorates. These are clear signs of a progression to myelofibrosis. Then the bone marrow is done.

Again, we don't have a way of predicting.  Follow-up of the patients at certain time periods is the rule of thumb. And if that happens, there are medications that we can utilize to control signs and symptoms of myelofibrosis.

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 September 24, 2014