Published on October 8, 2018
Myeloproliferative neoplasm (MPN) patients want to know what to expect from their condition over time, and how quickly and to what degree it will progress. What is the likelihood that essential thrombocythemia (ET) or polycythemia vera (PV) will progress to myelofibrosis (MF)? Why do some MF patients develop acute myeloid leukemia (AML)? Who is at an increased risk for developing another condition? Dr. Mark Heaney, from Columbia University Medical Center, explains the progression rates for MPN patients, and the role genetic mutations play in prognosis and risk assessment. Dr. Heaney also discusses other disease complications and practices for managing an MPN.
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Transcript | How Progression Rates Vary Among MPN Patients: An Expert Explains
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Dr. Heaney, tell us, what do we know about progression? Because people know, if it goes—blows through myelofibrosis, you could even have acute leukemia and maybe there’s some medicine changing for that now, but it’s very scary. So what do you tell patients today on what we know about progression and how variable it is?
So, I think the truth is that, most patients with polycythemia vera and essential thrombocythemia don’t progress. In fact, the progression rate to myelofibrosis is less than ten percent. So it’s—it’s certainly a concern and, as a physician, that’s my job to watch for that, but most patients don’t go on to progress. And so, there are other complications that can come with the disease and having a blood clot is probably the most common.
So that’s—that’s something that we can sometimes try to reduce the risk of and all those things are part of managing the disease in total. It’s—progression is scary and, you’re right, if you go to myelofibrosis, then there is that fear that there’s another layer of progression beyond that, but—but fortunately that’s not the case for most people.
Okay. So, we’re not necessarily on a freight train from ET through PV to MF to acute leukemia, so that’s good to know. So then it sounds like monitoring and the proper treatment for the condition you have. So with these ladies with PV then, what would be the monitoring you would be doing to lower their risk of complications from PV, since we have two ladies with it.
So the—the most common risk of PV is thrombosis or blood clots and we think that, over a patient’s lifetime, somewhere between a quarter and a third of patients with diseases like polycythemia vera and essential thrombocythemia may have blood clots. I—I think one of the things that’s really critical to remember and—and I know that you mentioned the—the life expectancy slide that was scary, but all of the data that we have right now is already 15 to 20 years old and this is a disease that extends over such a long period of time that the really knowing what’s modern and now is extremely challenging for—for us as physicians.
And so we do know that there is an increased risk of thrombosis, and we think that we can reduce that risk by giving medicines like aspirin in low dose, by controlling the hematocrit to keep it 45 and less, and in some cases, using what we call cytoreductive medicines, like hydroxyurea (Hydrea) and pegylated interferon (Pegasys) and possiblyother medicines like ruxolitinib or Jakafi.
So one of the things we have talked about before, Dr. Heaney, particularly related to myelofibrosis and some polycythemia vera, is genetic mutations that may be fueling your condition. Does that apply that in PV where you would be treating it differently based on that?
Not so much for PV. We think that, for patients for myelofibrosis and essential thrombocythemia, that patients who have the JAK2 mutation have a higher risk of thrombosis than patients who have other mutations, but nearly everyone with polycythemia vera has the JAK2 mutation, so that kind of makes everyone level, in that regard. And so there are other components to risk mitigation in that setting. We know that patients who smoke or have diabetes or high blood pressure have higher risks of thrombosis and so, working to reduce those—those risks and there’s certainly a value to reducing those risks for—for other diseases, too.