Progression of PV

[Buggerbear]

How do you know when your PV has progressed to MF?

[Otterfield]

You would have a bone marrow biopsy and your doctor can tell you from that. I think it's best not to try to guess from symptoms, as they can be misleading.

[Buggerbear in reply to Otterfield]

Thank you Otterfield......I'm sure my blood work would indicate low percentages also.

[Kari1961]

I agree with Otterfield - I had a conversation with my Haematologist a few weeks ago about allele burden. He said " We monitor you bloods every 6/12 weeks and would notice any significant changes and act accordingly if it looked like you were progressing from PV to MF.

I mentioned allele burden to him as I had no idea what it was, and rather than search Dr. Google and receive lots of confusing and in some instances misinformation, I would ask him. He gave me a thorough explanation in a simple-to-understand way and reassured that they thoroughly check all blood levels.

[Buggerbear in reply to Kari1961]

Kari, could you share that simple-to-understand explanation with me? Thanks so very much!

[Kari1961 in reply to Buggerbear]

Of course - Allele Burden is the percentage of the JAK2 (V617F) mutation we have in our bone marrow. A higher allele burden would mean progression into MF. Hope that helps.

[Buggerbear in reply to Kari1961]

Thank you Kari1961....that makes sense to me. My level of JAK2 mosaicism in 2014 was 10.9% so I have no idea of what it might be now.

[Dodders]

Hi there, I can only speak for myself but after 12years with PV and my Hb being kept under control with Hydrea I became anaemic. The bloods did not indicate any MF i.e. blasts and so the suspicion fell on some sort of internal bleed, e.g. a stomach ulcer. When this was ruled out I had a bone marrow aspiration and this revealed fibrosis. By then my spleen was beginning to increase as well, and after a referred consultation with an expert MPN specialist MF was confirmed. I had also noticed night sweats and generally feeling a little less well than before. Any change in symptoms and bloods should mean having a bone marrow biopsy performed. Even if no fibrosis is detected the test will provide a useful benchmark by which to assess any future changes when tested again. Good luck.

[Buggerbear in reply to Dodders]

Thank you Dodders!

[Cja1956]

I agree with Dodders. I had ET Jak 2, for about 10 years when my hemoglobin levels started dropping and I felt more unwell as I usually felt. When I suggested to my hematologist that maybe I had progressed, and maybe I need another BMB, he said he didn’t think so. So I found an mpn specialist, I brought the last 6 months of my records to her and my original diagnosis. I had a BMB, an ultrasound, and tons of bloodwork, and she confirmed myelofibrosis, intermediate 1. My hemaglobin was about 8.9 last year, and is now in the normal range, at 11.9. Good luck. This site gave me the courage to seek a second opinion and I never looked back.

[Buggerbear in reply to Cja1956]

You are lucky to have a MPN Specialist and I am so glad your hemaglobin is back in the normal range!! Good luck to you also and yes this site does give one courage! I appreciate all of you !!!

[Mustang50 in reply to Cja1956]

How did you go about finding a specialist? So grateful to everyone on this site so helpful!!!

[Cja1956 in reply to Mustang50]

Sorry, mustang 50. I’ve racked my brain to try to remember what site I used. I think I got it from Hunter back then so he would know.

[Cja1956 in reply to Mustang50]

Just found it. Go to mpnforum.com/list-hem/ and you will find specialists all over the world.

[Mustang50 in reply to Cja1956]

Thanks so much!

[tracey13]

My husband was feeling absolutely exhausted day the hxdroxy didn't seem to be working.His spleen was enlarged and a bone marrow biopsy should primary fibrosis.

He's now on ruxolitanib and feels so much better.

His blood levels didn't change to indicate primary PV. He still gets bloods every 3 months but has telephone appointment every 4-6 months while covid is around.

X

[Buggerbear in reply to tracey13]

Tracy, so glad your husband is feeling better!

I too am on Ruxolitinib, 15mg, twice a day. Think these meds work better than the hxdroxy for me.

[tracey13 in reply to Buggerbear]

Yes I agree makes me wonder if my husband was on ruxolitanib from the start of his PV when venesections weren't working enough would ruxolitanib stopped the primary progression to mf.He was due another bone marrow biopsy this year but with covid it's not been possible.

His consultant said soon as blood levels drop or change this is when we know we need to look into things again. So far so good bloods have been stable for a long time now .

Tracey

[hunter5582]

Others have already given you good technical information about what to watch for in terms of progression to MF. The MPN Specialist and my regular hematologist and I monitor my CBCs ongoing as well as all of the secondary symptoms. Any sign of a change in my baseline that would indicate a change in disease process triggers additional testing. Thus we just rechecked my JAK2 Mutant Allele burden and and the MPN Myeloid Panel for the first time. There is no indication of disease progression so no need to do a BMB at this point.

Re. the BMB, different docs have a different take on that. Some do it routinely at initial diagnosis. Others do not do it until there is an indication for it. The MPN Specialists do not all agree on this. Therefore, my take is that the patient preference should drive that decision. You can talk to your doc about precisely what can be learned from a BMB in your case. Then you can do a risk/benefit analysis of the BMB in your case and make your own decision. Both of my docs do not support doing the BMB in all cases, only when there is a specific reason to so so. I agree (for my case only). I will do the BMB the minute there is an indication that I should do so. I would agree that in any case where there is a question about diagnosis or any sign of disease progression a BMB is indicated.

Do note that hematologists do not agree on a number of topics from medication choices to checking things like allele burden and non-driver mutations. It can be rather bewildering to listen to all the different things the docs are saying re. MPNs. Gaining an understanding of the basic science that drives MPNs does help make sense of it all. The good news is that there is some emerging consensus regarding PV diagnosis, tracking and treatment.

LOL (at myself) for a long wordy answer to your question. The bottom line is to work with a MPN Specialist to track the trends in your labs and do additional testing when it is indicated. While progression to MF is possible, most of us with PV will not progress to MF or AML.

All the best.

[Buggerbear in reply to hunter5582]

Thank you Hunter for your information!! You always give a thorough explanation and I have learned so very much from your posts and those of others on this site,