Does anyone have ET/PV for more than 40 years without progression? I really haven't heard of any in this site..

[Aneliv9]

I read about many ET/PV progressions and i am curious if anyone has stable ET/PV for many many years without progression

[ritaandscooter1]

This is a great question! I am 61, PVjak2 and have had PV for 20 years. My treatment has been only aspirin and Phlebotomies during this period of time. Have tried PEG briefly but my liver couldn't handle the drug on the standard low dose and the side effects were not good. My MPN specialist recommended 2 aspirin a day and phlemotomies as needed which have been every 4 to 5 months. This regimen has worked well for me combined with daily exercising. My Hematologist wanted me to get a bone marrow biopsy because it has been over 19 years since my first one and he wanted to check for progression. I'm very HAPPY to say that there were NO signs of progression after 20 years and with a AB of 93%. Here are some of the direct notes written from my hematologist after receiving the result of the BMB taken on 8/1/22.

*No increase in reticulum fibrosis

*No increase in myeloblasts (0.8%)

*Bone marrow cytogenics are normal

*No dysplasia in any in any of the cell lineages

*Trilineage hematopiesis increase in megakaryocytes without dyplasia

*I will be getting the NGS genetic panel back around 9/2 so will add that data as well.

*Note: pretty bad iron deficiency which explains why my HCT wasn't rising as normal. I'm taking very minimal low dose iron tablets but it is a balancing act.

*Besides iron deficiency I have not had many symptoms of PV. Occasional itching when temperature gets colder.

Best wishes to all..........Kerry

[Meatloaf9 in reply to ritaandscooter1]

Sounds like an excellent result, please let us know how your NGS comes out. Best to you.

[EPguy in reply to ritaandscooter1]

That is so good to feel well. Wishing you many more years of that.

"No increase" in fibrosis means you have the normal amount, I recently posted about that, everyone has at least some marrow fibers to support the marrow, and yours remain like that.

In this part "Trilineage hematopiesis increase in megakaryocytes..." I take it to mean there was no increase in Trilineage hematopiesis, only in the megakaryocytes. But the wording is a bit ambiguous.

It says "Bone marrow cytogenics are normal" It seems your Jak2 should show up there, but this may be measuring something else. My marrow lab had Jak2 here.

I think no-dysplasia means your marrow is making normal blood, even if in abnormal amounts. I'm trying to learn about this in another thread.

[ritaandscooter1 in reply to EPguy]

Thanks EPguy, These were some of the notes from the BMB that my hematologist had written in summary. I'm still trying to understand as well the medical terminology. I'll request a copy of the actual BMB report at my next appt. on Sept 2nd. and should have the NGS genetic sequencing report as well. Maybe the actual report referring to the "Bone marrow cytogenics are normal" will have more written regarding the Jak2 mutation.

The "no dysplasia" means there's no abnormal growth or development of abnormal cells although there is an increase in megakaryocytes (lots of platelets cells) I have hovered around 1000 since being diagnosed.

Best wishes to you. Kerry

[william-Indo in reply to ritaandscooter1]

High AB is good...?

I got BMB 2mo ago and AB is 91%.

[ritaandscooter1 in reply to william-Indo]

Hi William, to answer your question about high AB, my hematologist said there are basically two views of thinking regarding the importance of AB counts. The jury is still out on whether high AB counts lead to progression and more symptom burden. In other-wards if you go to MD Anderson they tend to believe AB plays a significant role. I see a MPN specialist out of Mayo in Rochester who clearly does NOT find any definitive significance to AB being a factor as of yet. He has published many articles and researched the role of AB with MPN's. He basically told me his mind can be changed if there if verifiable scientific proof that lowering the AB makes a difference. Both my hematologist and MPN specialist share this same view. My AB should be high as I have not been on cytoreductive medication to lower them. Perhaps I am one case study in which a high AB has not contributed to progression after 20 years. There are so many factors though involved with progression besides AB count such as other driver mutations. I'm awaiting my NGS genetic results which will interesting. Are you on any medication to control your counts? Kerry

[william-Indo in reply to ritaandscooter1]

Thanks Kerry

[monarch5000]

20 years after diagnosis:

85% of PV patients who had been treated with interferon had not progressed to MF

59% of PV patients who had been treated with hydroxyurea had not progressed to MF

51% of PV patients who had been treated with phlebotomy-only had not progressed to MF

95% of PV patients who had been treated with interferon were still alive

63% of PV patients who had been treated with hydroxurea were still alive

57% of PV patients who had been treated with phlebotomy-only were still alive

Source: tinyurl.com/2p8z23b8

[MPort in reply to monarch5000]

Thank you for posting these. As my grasp of statistics is vague could you explain the relationship of the lack of progression and the death numbers. So take interferon as example. 85% with PV/interferon have not progressed. 95% PV/interferon still alive.Many thanks

[Manouche in reply to MPort]

The main cause of death is thromboembolic, not progression. That’s why you see this discrepancy between death and progression on statistics.

[monarch5000 in reply to MPort]

"the excess deaths from the progression of PV disease to myelofibrosis can be prevented with interferon alfa. In fact PV patients treated with interferon [for 20-30 years] had a normal life expectancy across a 30 year duration of follow up." youtu.be/W3JJBS645c4(Not working as expected?)

[Aneliv9 in reply to monarch5000]

But this normal life expectancy doesn't exclude the possibility that some of the PV patients were already at the PV/MF stage. Because they were still alive. Am i wrong? (Hope i am!)

Also 15% rate of progression at 30 years, is quite a bit..

[EPguy in reply to MPort]

This post from last year has some additional info and details on the long term IFN studies

healthunlocked.com/mpnvoice...

[Otterfield in reply to monarch5000]

As far as I know, interferon wasn't an option 20 years ago, at least in the UK. Perhaps it was in other countries.

[EPguy in reply to Otterfield]

The long term studies were done by Richard Silver's group at Cornell, some of their work dates to the '70s. See the link above to last year's post on these. This is long before pegylated IFN, so the good results they got are impressive. They have been promoting IFN benefits for a long time, the field is only recently comfortable joining them.

You're right that few if any other clinics were using IFN so long ago.

[monarch5000]

Of the patients that took interferon for 20 years, 15 % progressed to post PV MF and 5% of those that did progress ended up dying during that time frame.

Of the patients that didn't take interferon, a much larger percentage progressed to post PV MF and a much larger % of those that did progress ended up dying during that time frame.

[beetle in reply to monarch5000]

The way that is presented infers that the deaths related to MPN progression. Without further information on the patients’ age, other complications and cause of death this is possibly leading the reader to the wrong conclusions

[EPguy in reply to beetle]

You're right that they claimed progression improvements. This chart is from one of their studies. In the lower left plot the high blue line is the large improvement for MF free survival via IFN. Lower right in over all survival improvement.

I think the smaller advantage of HU vs plb may be with improvements to thrombotic events rather than progression.

LongTerm INF

[kevmince]

I am 52, with ET ( Jak2) , I have been on hydroxy about 24 years since diagnosis, and historic blood tests suggest I had ET at 16, my last bmb had no progression 4 years ago , so if my maths is correct that 32 years plus without progression, bloods are still stable , so make of that what you will.

As mentioned above , be wary of the stats , because mpns are far more likely to be diagnosed in older people so mortality rates are bound to be higher , and those of us that were diagnosed 20 plus years ago weren't offered interferon , and in my case since hydroxy keeps me stable we have taken a " if it's not broke , don't fix it" line

[Aneliv9 in reply to kevmince]

I believe i have ET at least since 17 (now 39). Do you take interferon??

[Otterfield in reply to Aneliv9]

Were you diagnosed at 17? If not, why do believe you had it at that age?

[Aneliv9 in reply to Otterfield]

I was officially diagnosed 3 years ago. But i have had high platelets at least since 17 years old. I have not kept blood tests before this age.

[kevmince]

No I have been on hydroxy the whole time , and since my bloods are stable on it haven't tried anything else . I was diagnosed before interferon was offered

[georgevanier in reply to kevmince]

So good to hear Hydroxy work so well for you, will be start this med soon, what time of the day or night do you take it, with or without food. Thanks for your help .Heather

[kevmince]

I take mine in morning , just after breakfast

[Chicagopv]

I've had PV for almost 20 years. Was on hydroxyurea and 3x/week UV light therapy for extreme itching. I switched to Jakafi, which really helped the itching. It's very expensive in the US if you're on Medicare (as I now am). I also take Atarax/hydroxyzine for excess itching. I get red when I'm hot, I sweat a lot, can't stand being in heat. I itch a lot when it's hot and there are allergens about (pollen, mold). I love winter.

[ainslie in reply to Chicagopv]

HiDid I read that correct that you were on HU and UV light therapy for 20 years for itch?

I was on UVB for 10 years , 7 of those years on venisection only and the last 3 years of UVB I was on a combo of RUX and venisections, now I am on Rux only , no UVB or venisection. I tried to avoid taking HU because I was concerned about developing skin cancer while also doing UVB daily. Did you have any problems with that combo and skin cancers. Have you tried beta alinine for itch, I haven’t but heard it’s helpful.

I was an Olympian itcher , UVB and exercise helped but Rux was a game changer. I am on 20mg+17.5mg for PV,I still have mildish itch after shower but very doible

[Aneliv9]

Until now no-one has the chronic phase of MPNs for more than 40 years

[EPguy in reply to Aneliv9]

To be honest, at 40 years the odds are not great for us. Esp as most are older when Dx. You can see in the plots above in my reply that even at 30 years with the best treatments it gets iffy. But patient's age as noted above naturally makes a big difference. I'm doing well in therapies, but I do expect odds favor some reduction in my life unless we get a "cure" as Hepatitis C recently did. These patients don't normally need IFN anymore.

Here is one study with data on young vs old MPNs:

<<Median survival in young patients was 37 years for PV, 35 for ET and 20 for PMF;

the corresponding values were 22, 22, and 8 years for ages 41-60 years and 10, 11, and

3 years for ages >60 years (P < .001)>>

pubmed.ncbi.nlm.nih.gov/301...

So this does get near that magic 40 years survival for under 40's. But there are many above and below the median, so these numbers are not what most will actually experience. Note this study dates to 1967 so we would expect and do see signif longer survivals with modern treatments. I don't expect to expire in the median 8 years predicted here.

[Aneliv9 in reply to EPguy]

I was more curious to know if someone has ET/PV without progression. Maybe you answered me this above in a manner and i didn't get it. Cause i have ET for more than 20 years already and i am 39. So in 20 years from now is inevitable that I will have MF?

[EPguy in reply to Aneliv9]

I can comfortably say with fact for us with ET or PV progressing to MF through our remaining lives, however many years that is, is not inevitable.

According to the plot earlier in this thread, after 20 years, with the highest risk therapy, odds were about even to stay MF free. With the best therapy odds were about 85% to stay MF free. That does leave a 15% probability of acquiring MF. (reminder, these are not in any way predictions)

I've learned thru the forum and research how incredibly variable our experiences are. We can study our risk factors and consider the best and latest with that, but for us there is always an unsatisfying level of randomness no matter what we do. Because of this I do understand your frustration with the uncertainty that hangs on us, but I feel as long as I am getting the best avail therapy for me and I'm feeling sort of ok, all is good.

[Aneliv9 in reply to EPguy]

Yes but 15% at 20 years is very high percentage! I mean what will be my percentage in 40 or 50 years if i am now only 39 and have ET for 20 years already??? I just wrote a new question in the forum with a chart of progression and I want someone to explain this.to me!!