Best treatment for Post PV/MF ?

[MCW22]

I'm wondering Which medication is more effective for post pv/mf. Ruxolitinib or interferon?

I was diagnosed with pv in 2017. 2 years of hydroxy, then ruxolitinib at 20mg a day. A bmb in december showed progression to mf. Grade 2 fibrosis. The rux has recently been increased to 30mg a day along with 1 iron tablet on alternate days. My hb had increased to 106 as a result of the iron tablets at my last blood test, and the white count had gone down from 42 to 38 It will be another 6 weeks till my next test which will be the one to reveal if the rux increase has made much difference. I've been given a prognosis of between 18 months and 3 years. Maybe more, maybe less were the words of my consultant.

Naturally I'm wondering if I'm on the best treatment as I keep reading that interferon reduces fibrosis. I don't get any side effects from rux so I'm also wondering if its a case of better the devil you know. I'd like to know what medications others with post pv/mf are on in the UK.

[EPguy]

I recall your recent Dx, wishing you the best.

In this report from the Silver group "Intriguingly, (IFN) treatment seems to work best on patients with early stage MF and, in this setting, interferon therapy can even reverse bone marrow fibrosis" So which stage MF you're in could be part of the decision. This suggests starting without delay if it's considered an appropriate option.

silvermpncenter.weill.corne...

Do you have access to the newer Jak inhibitors? ie Ojjaara for anemic pts with MF. It was just approved in US so maybe not yet in UK.

[MCW22 in reply to EPguy]

Momelotinib has just been approved in England but from what I've read it's not for people who have already been on a jak inhibitor. I've been on ruxolitinib for a few years now. I did mention it to my consultant but she didn't say anything, more or less ignored what I said. I will see what my bloods are like at my next appointment in 6 weeks and maybe query inteferon and see what she says, although I don't like the sound of it's side effects.

[beetle in reply to MCW22]

As far as I know there is no problem taking Momelotinib if you have been on a previous Jak inhibitor. I have taken Ruxolitinib for many years and am considering switching to momelotinib at the suggestion of prof Claire Harrison

this is all best discussed with your MPN specialist

Best wishes, Jan

[EPguy in reply to MCW22]

That would be surprising if Rux use precluded use of other Jak-i's since Rux intolerance is a key reason they were developed. Maybe it was a limitation in some of the trials?

On IFN sides: Jak-i's and IFN both have side effects as do all meds. Rux tends to have more, (weight gain is a top minor one) IFN tends to have potentially more serious. As you've likely seen I have been devastated by one of the worst case IFN effects. But IFN for most gives plenty of warning for these; I did not have this benefit but the odds of my horrible line up are really small. In your case the possible upside is worth discussing with Drs.

[MCW22 in reply to EPguy]

The fact that I don't get any side effects whatsoever with the rux makes me wonder if I should just take my chances with it. I dare say the increase in dose will help. In the words of Ann Tayler, We're all gonna die someday lord, we're all gonna die someday 🎵

[EPguy in reply to MCW22]

Same here, Rux has been entirely boring, even the pill is a cheap chalky thing. With Bes, even when all was great, it politely reminded me I was taking it, as seems common here.

A main reason you might consider the newer Jak-i vs Rux is if anemia becomes more trouble.

[hunter5582]

It is rather hard to compare ruxolitinib to interferons in your situation. That is really a question to be reviewed with your MPN care team in light of your specific MPN profile. It is worth noting that NICE approved momelotenib for MF with anemia. You might wish to discuss this option with your care team. healthunlocked.com/mpnvoice...

[monarch5000]

Interferon, or ideally, low doses of both combined together: ncbi.nlm.nih.gov/pmc/articl...

[Scaredy_cat]

Given your prognosis has a stem cell transplant been considered?

[MCW22 in reply to Scaredy_cat]

I was told it was not advisable due to my health history including previous stroke and 5 spinal fractures, and having read up about it I don't think I would want to.

[marlenablue]

I have post-PV MF and not on any treatment at this point. Interferon lowered my platelets too much which led to the BMB that diagnosed me as MF grade 3. My blood counts are stable at this point though I do need phlebotomies occasionally. I take baby aspirin and that is it. As others have said, the best treatment for you should be discussed with an MPN specialist. Best of luck and don't let the prognosis get to you - there are treatments now and in the pipeline for MF. Hang in there!

[Cranston3x in reply to marlenablue]

Hi Marlena,

What do you you mean interferon lowers your PLT too much that led to BMB diax as grade 3 MF? You mean by lowering too much PLT can cause more fibrosis in the BM? Or you have progressed and the interferon didnt help much yet?

Thanks,

Steve

[marlenablue in reply to Cranston3x]

All my blood counts lowered due to the Besremi but the platelets were severely low - below 100. The results were so drastic that the doctor wasn't sure if it was due to Besremi or to other issues like possible MF. I had other risk factors for MF - enlarged spleen, increasing wbcs and the SF3 mutation. This is why I had the bmb and diagnosis of MF. Interferon is supposed to lower blood counts but I guess not that fast or dramatic? To answer your question, I had progressed to MF but not because of the interferon.

[Cranston3x in reply to marlenablue]

Hi Marlena,

I am sorry you are diax with MF3. Did you just get treated with Besremi for PV and then suddenly because of the lower PLT, you were due for BMB to find out you got MF? And are you considering ASCT?

Thanks,

Steve

[marlenablue in reply to Cranston3x]

Your summary is correct. I will consider an ASCT when the time comes. Right now my blood counts are stable, pretty much the same since 2016. They returned to my 'normal' after the Besremi was stopped. I even needed a phlebotomy in Dec due to a high hematocrit. My doctor says I am low risk right now and has not given any timeline or prognosis. I have no symptoms except what shows up in my CBC. I am 53 .

[tracey13]

Hi,

I don't think you should of been given a lifespan as long as your post PV MF is managed you can live a normal life .

My husband has been on ruxolitnib since 2019 . All his bloods are in normal range he's had PV since the age of 40 he's now 51

There's people on here had PV/MF for over 30 years or more .

Tracey

[MCW22 in reply to tracey13]

I too have been on rux since 2019, however my white count has never been in range, peaking at 48 which prompted my second bone marrow biopsy revealing the change to mf. It also shows 6% blasts. My hb dipped to 92 but with an iron tablet every other day it has come up to just over 100. I am getting more spleen pain recently too. I'm now taking 15mg in the morning and 15mg in the evening so hopefully this increase will help.

Carol

[tracey13]

My husband takes an aspirin a day and 20mg morning and night of ruxolitnib.

He last had a BMB in 2018 which revealed post PV primary fibrosis MF. It was really worrying at the time but in all honesty ruxolitnib is doing the job .

We don't think about it as much now .

He Has blood tests every 3 months and a 6mthly telephone review with his consultant.

Tracey

[MCW22 in reply to tracey13]

Would you mind if I ask what his blood numbers are?3 months seems a long time between appointments. I've never gone that long. Recently been every 4 weeks, but had to leave a longer gap this time as I couldn't get to hospital when they wanted me to.

Carol

[tracey13 in reply to MCW22]

Hi, My husband never ever gets the blood results .

His consultant always says if there's any change he will contact him.

He's got a telephone appointment begining of next month.

He used to have venesections weekly this lasted for weeks then it went to fortnightly.

Eventually he went on hydroxy for a few years this absolutely drained him and wiped him out day to day was a real struggle.

He had fortnightly bloods for two years when he first went on ruxolitnib then went on to monthly . Then two monthly and now it's every three months .

He's been monitored since 2019.

There's an MPN app you can input your blood results it's really good app.

Tracey

[hunter5582 in reply to tracey13]

Never ever getting lab results is not acceptable. The information in the medical record is your husband's property. it is his legal right to access this information upon request. Depending on which system he is in, he may be able to access his labs directly through a patient portal. If this is not an option, then he just needs to formally request the lab results. Note that electronic medical records can usually print a historic view, showing the lab results over time. This is often to most useful way to look at the labs.

Wishing you and your husband all the best.