I am 57 and was diagnosed with PV Axon 12 about 2.5 years ago. I live in US. I am in perfect health otherwise. No heart disease , low blood pressure, no diabetes. normaI cholesterol, zero calcium score. No surgeries. I am very physically active and in excellent shape. I take no medication except daily baby aspirin.
My Axon 12 mutation has been extremely mild. My platelets have hovered around low 600’s. Otherwise all other blood work normal. I currently take a baby aspirin daily and go for phlebotomies every 2.5 -3 months, I take daily supplements, eat a Mediterranean diet and exercise daily. I have no side effect from the aspirin or the phlebotomy. I currently am being seen by 2 hematologists and 1 MPN specialist from Moffitt in Tampa, Fl. I was also seen by another MPN specialist because he was doing a research study on Resurfimide. He wanted to see me if I was a candidate. He pulled out a chart and said based on my symptoms and condition, that my life expectancy was close to 30 years. I feel lucky to have an extremely mild case. I have not had a BMB as we have discussed, but doctors have not pushed for.
All of my doctors do not want me consider changing or doing anything until I hit the magical 60 years of age. And, even then we could wait and see?
My question, based on my current history should I consider 1. Besremi for its potential molecular remission, ( I am also concerned about side effects considering I have none now) 2. Be happy that I have a mild case and wait and see.( I am aware that PV can be a progressive disease, but my doctors tell me stories that they have patients with PV who are in their 90’s doing quite well and have outlived 5 hematogists).
Any thoughts would be greatly appreciated.
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FlTodd
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Some of us with PV do have relatively indolent cases. PV can be quite variable and there is no "one-size-fits-all" approach to care.
The protocol for PV does call for cytoreduction at age 60, when we are considered to be high-risk based on age. This is a valid statistical projection for groups of people with PV, but may not always be a valid projection of individual risk. We each need an individualized assessment of risk. We need to consider our treatment goals, risk tolerance, and treatment preferences.
If you prefer to continue your current treatment strategy based on all of the above, that is a reasonable decision. You doctors are obligated to advise you to consider cytoreduction based on the widely accepted PV treatment protocols. It is up to you to decide when the benefits outweigh the risks of initiating a different treatment plan.
I did not base my decision to initiate treatment with the interferons on age. I was about to turn 66 when I started on Pegasys (later switched to Besremi). I was following a phlebotomy-only treatment plan at that point. What I found was that the long-term adverse effects of phlebotomy-induced iron deficiency were worse than the PV symptoms. I also decided that pursuing a reduction in allele burden was in my best interests, particularly because I have an additional non-driver mutation (NF1) and several co-occuring conditions. I am very glad I made this decision. My quality of life has improved on Besremi and I achieved a reduction in allele burden from 38% to 9% in 18 months of low dose interferons. I hope to achieve a deep molecular remission.
Hopefully, Rusfertide will soon be available for people with PV. It should prove a very useful addition to the treatment options along with Besremi and Jakafi. Perhaps it will be available when it is time for you to make your choice at age > 60.
The huge advantage of starting interferon now, instead of waiting a few more years when your malignancy is more advanced, is that only low doses that have few, if any, side effects are more likely to be effective.
The reason you often hear interferon users on this forum complaining about side effects is because they started the drug late so larger doses were needed.
I'm not a doc, but I would consider your PV already fairly advanced given your platelet count is up to 600 and you need phlebotomies 4-5 times a year. So even if you started interferon today it might take 1, 2 or even 3 years before you became phlebotomy free.
I would suggest asking for Pegasys interferon instead of Besremi because Pegasys is 4 times less expensive, hence less worry about affordability. It's also available in vials that enables the patient that wants to save even more money to get 2-4 doses out of each vial.
In the USA, the cancer center with the most experience treating with interferon is in New York City and here is a very brief video of one of their docs emphasizing the importance of early treatment: tinyurl.com/2vr66w2s
Unfortunately being symptomless is not an indication for how the disease will behave in the future. I asked Dr. Ruben Mesa at a patients conference whether my being symptomless (with a PV diagnosis) meant that I was unlikely to progress and he answered that the disease can change regardless. I was 58 and had been diagnosed that year. Now I am 61 and found out a year ago that my JAK2 VAF was 79% and have been on interferon since. I prefer to do what I can now whilst I am still fit and strong (so to speak).
Hi. I agree with the people responding above. Lack of symptoms is not always an indicator of the disease severity, but your body’s abilities to handle your blood changes. Also some mpn docs and researchers are questioning the age related low risk /high risk treatment approach. In fact, phlebotomy alone was looked at as a treatment and it scored lower in long term outcomes than the various drugs. See Dr Silver and Weill Cornell studies as Monarch5000 showed above.
I was diagnosed with PV exon 12 almost 3 years at age 67 with no symptoms. After 2 years on pegasys interferon my dose is a quarter of what it was - some imperfect indicator of a reduction in disease severity. People have different tolerance for the interferons- I have tolerated them well. Phlebotomy also will introduce iron deficiency issues at some point which for some is a severe side effect. Interferon has been shown to reduce disease burden which would seem to be a good thing no matter what your age.
Congratulations on being symptom free, I wonder what age you are now and if you have had your allele burden measured.? You may have mildish PV but 2.5 to 3 venisections is fairly normal PV . 2.5 years is early days and things can change , it’s often a good idea to get a BMB and allele burden number or even better add NGS as a baseline, then you can repeat these tests periodically to help monitor any changes. You may stay as you are , I hope so but I know veterans who have had MPN 30 plus years and they say don’t assume anything or turn your back on your MPN because for some it can change quickly. I would love to know how your doc came up with the near 30 year bit , I am a bit sceptical , don’t limit yourself to that , I know of some over 40 years and don’t forget meds are changing so who knows what’s 5-10-20 years from now in terms of treatments or hopefully cures. To drug or not is big decision, they will tend to put you on meds at 60 anyway, you are wise to research all that carefully and if it were me I might consult a second expert re drugs or not.
I wouldn't worry about life expectancy with PV (or ET). In any case, for a 57 year old that would be pushing 90! I think it would be worth asking about interferon, though bear in mind that it can have some unpleasant side effects, though not for everyone.
Besremi just got approved in July 2023 for low risk PV if that tells you anything. I have been on it 6 mos and seen huge improvements. And I have always been asymptomatic and only needed phlebotomies maybe every 5 mos. Of course, I have other health issues and I am 41. So, progression has always scared me since hopefully I have a ways to go. But I think you need to discuss pros and cons with your doctors. My PV didn’t cause any symptoms but I had underlying MS and didn’t know it til a year ago. And my two diseases are related somehow and seemed to affect one another. I am currently on 200mcg of Besremi without side effects in fact I feel better on it. We are doing the slow adjustment route. MD Anderson doctor said she thinks I will end up at 250mcg but is ok with only increasing once a month. I go in a few weeks for labs. And then she said once we get to maintenance phase maybe 300mcg once a month. I hope maybe this helps start a conversation with your doctors. My platelets dropped from 1.6 million to now 500. And fingers crossed no more phlebotomy for me for the rest of my life. Good luck!
At a minimum you need a baseline bone marrow biopsy to really see what is going on. As others have said, it's great that you have no symptoms and no comorbid conditions but that does not necessarily predict how PV will progress. What is your allele burden?
The Mayo doc I consulted had me do additional genetic testing to see if I have other predispositions to my PV progressing to something worse. That's another test to put into use for decision making. I am 65 and had 6 injections of Besremi which brought down my HCT very well (you don't mention your HCT numbers?) and my platelets pretty well. My liver enzymes and economic concerns meant I had to pull off the medicine. I'm interested in returning to it at low doses only, but my application to the Pharma Essentia company for financial aide has moved ridiculously slowly and arduously. The were concerned my household income was low enough to put me on Medicaid or get extra Social Security help for drug costs. (I am not yet taking social security, or any other retirement income yet.) I assured them that my investments and home owning put me squarely in the middle class with no access to those safety nets. Still, they dragged their feet and then asked me to file two enormous federal and state forms that turned out to be impossible--SS form would not take my numbers and the other was so detailed (date of each dividend received, date of car purchase, etc etc) as to increase my risk of stroke right there and then. I sought help from my clinic, got conflicting financial numbers from Medicare, and finally wrote a letter to the Besremi bigwigs to tell them that the process was not reasonable. Now if you have regular but modest income or fabulous insurance, you might be ok paying for Besremi. I may have to ask for its alternate, Pegasys , as my platelets have risen precipitously while waiting for financial clarity.
It depends on which test is being done. Generally, genetic testing can be done with blood. In addition to a JAK2 quantitative analysis, I have had the Intelligen MPN panel done.
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