Pre Fibrotic Myelodibrosis

[kiwitraveller]

Just wondered if there was anyone else in this group who has been diagnosed with prefibrotic Myelofibrosis . It was only discovered in 2016 and when I found I had it my platelets were 1600 and I had a low amount of fibrosis in my bone marrow. Apparently it may or may not develop into full Myelofibrosis. I have minimal symptoms. Apparently it is pretty rare.

[katiewalsh]

Hi, I just learned this spring that my MPN is Pre-fibrotic MF. I’d always thought I had ET or MPN unclassified. Do you know what grade of fibrosis was found in your BMB? My only BMB was in early 2014 & mine was level or grade 1. My spleen is enlarged but not grossly so. I do know it may or may not progress but didn’t know it’s rare. Do you recall where you learned it’s rare? I’m interested in seeing how many others on here have it. Great to know you’re doing well! Katie

[kiwitraveller in reply to katiewalsh]

Hi Katie

I am also no 1 on the fibrosis scale. My spleen at the moment is normal so fingers crossed. I am a Londoner but went to a conference in Oxford recently and of all the attendees I was the only one with Pre fibrotic. It is probably rare because it is unknown and people may not have been diagnosed but I found some info online about it - there is not a lot. My disadvantage is that I have Jack 2 plus 3 other mutations, but what to do - I feel fine.

How have you been feeling?

Alison

[katiewalsh in reply to kiwitraveller]

Hi Allison. Sorry I’m late replying. Until last year I felt pretty good but then my fatigue became a big problem. Fortunately my husband has been able to take over most of the shopping, cooking, etc. I can only do one thing each day or I get too exhausted. My spleen has been slightly enlarged for a few years but I’m not sure if it’s gotten worse. I’m glad you feel fine!, someone else posted in this thread about PMF but I believe that’s Primary Myelofibrosis, not pre-fibrotic MF. Let me know plz if you find anything interesting about what we have. Be well! Katie

[kiwitraveller in reply to katiewalsh]

Definitely will keep looking out for more info on it

Take care

Alison

[socrates_8]

Hey there Kiwi Traveller...

I am another antipodean from just across the ditch... (Sydney)

I was initially diagnosed as ET Pre-fibrotic, and was eventually classified as Post ET / MF.

However, that does not mean that everyone is the same, because of course... we are not. We are all just a tad dissimilar, and that can make a world of difference...

For instance, my first BMB was Grade2, which is quite a lot of scarring to the bone marrow, (fibrosis).

I also have ASXL1, which is considered a High Risk (HR) mutation that generally speaking, is supposed to mean a more adverse outcome...

However, as many others here are aware about me... My last BMB saw a significant molecular reversal, which revealed a reduction in my fibrosis from Grade2 down to Grade1.

Personally, I like to believe that much of that good fortune is due to my newly developed anti-inflammatory diet, intermittent fasting, and of course my cycling regime...

So much so, that I started an Australian FORUM, and an MPN Fundraiser to help bring much greater awareness to people from all walks etc.

mpn-mate.com/forum/viewforu...

This time next year, I will set off to circumnavigate Australia by cycle to raise funds for MPN Research to be conducted here in Australia.

Please feel free to stop by and take a look...

mpn-mate.com/fundraising-fo...

I was also diagnosed in 2016, and my platelets at that time were 1.7M. I have also been visited upon by two (2) TIAs, (minor brain strokes).

Nevertheless, I am determined to do this RIDE, and raise both awareness and these much-needed funds for more MPN research. Naturally, I am eternally optimistic... 20,000kms in Under 200 Days... is a very, very long way...

Best wishes

Steven

(Sydney)

[kiwitraveller in reply to socrates_8]

Wow impressive. I have 4 mutations but like you work out 4 times pw and am pretty close to eating a vegan diet which I think has helped keep things under control. I go to Sydney each year as my daughter lives out in MacArthur so would be good to catch up with your group.

Will keep an eye on your cycling progress good luck that will be a challenge, good on you.

I live in London but travel to NZ and Aus for 2-3 months each year although this put a stop to my trip last Xmas as they wanted to get my bloods stable.

[Aneliv9 in reply to socrates_8]

Your first BMB with fibrosis grade 1, was a diagnosis of ET? And what medicines do you take?

[kiwitraveller in reply to Aneliv9]

Hi I take daily asprin and 7x hydroxy a fortnight. This seems to be slowly reducing platelets- now 580 - and keeps symptoms at bay with no adverse effects.

[kiwitraveller in reply to Aneliv9]

As to the diagnosis my first and only has been Prefibrotic Myelofibrosis. I did not receive a final diagnosis until after my BMB

[Aneliv9 in reply to kiwitraveller]

Ok i understood now. Thank you for answering me

[Stephen399b]

I was diagnosed with ET Jak2+ in 2016. After the BMB they said there were signs of PFM and the person assessing the core sample went with this, whilst the haem went with ET. I also have systemic mastocytosis, just to make a complete score at Scrabble!

Spleen ok, platelets have been ok for about 2 years on HU x10/week, but are beginning to creep up over 400, so may need another capsule.

I don't know how rare PFM is compared to the other MPNs, none of them are common. My Haem (second one and much better than the first - listens to my concerns) said that they would treat ET and PFM the same anyway and a second BMB last year showed no extra scarring.

I am 68 and still work pretty much full time, but do get tired. Apart from that I am lucky that I can keep cracking on.

Best wishes to all

Stephen

[kiwitraveller in reply to Stephen399b]

Hi Stephen

You are the same age as me and I am managing really - have virtually no symptoms and am on 7 HU a fortnight. Platelets currently 580 down from 1600 but gradually moving down so they are keeping me on this dose which is great.

I was told they only discovered PMF in 2016 and a lot is not yet known about it but the treatment is the same as other MPN’s

Have always had a healthy diet and kept fit so am moving to a vegetarian/vegan diet now as it is supposed to support inflammatory diseases - worth a try and I work hard at keeping fit to keep any other illnesses at bay.

All the best

Alison

[Timjonze]

I think there is still some debate about whether pre-MF deserves its own distinction, hence why you are likely to get different diagnoses. I was give a definition of pre-MF by my first pathologist and ET by the next (my platelets are around 500 but spleen is a big 21cm). My current haematologist believes that ET can express itself at various stages of progression but that this doesn’t matter as much as the pace of progression. So pre-MF might not develop into MF for a long time in some patients, while ET could develop into MF quickly in others. Have you had any genetic testing to see what other mutations and risk factors you have? Might be worth enquiring. But don’t worry too much about the diagnosis term itself. Like you I have grade one scarring but my docs say the chance of progression is no greater than for anyone else with “standard” ET. Good luck with everything.

[kiwitraveller in reply to Timjonze]

Thanks for that - have Jak2, CALR plus two other mutations but Haem says nothing to worry about! They calculated my life span at 13 years with a 50% chance of swinging either way. I have no other conditions and my spleen is fine. 13 years is fine as will take me to 80 so not considering Bmb less would be 😩 but as am extremely healthy and working on it all the tjme hope it swings the other way.

[Aneliv9 in reply to kiwitraveller]

What do you mean by swinging either way?

[kiwitraveller in reply to Aneliv9]

13.5 years either - or + 7 yrs

[Aneliv9 in reply to Timjonze]

I am just like your case, spleen enlargement 15cm, bone marrow MF1 and platelets 470. My heam diagnosted me as ET. The same was mentioned in my BMB results. Did your heam told you what your pace of progression looks like? Also what chance of progression gave you?

[kiwitraveller in reply to Aneliv9]

Yes 13.5 years with a 50% swing either way but I have 4 mutations. I have been diagnosed as prefibrotic Myelofibrosis by Guys and have a lot of faith in them

[Aneliv9 in reply to kiwitraveller]

I see.. what is the plan in the worst case senario? Just try to understand what doctors do.

[kiwitraveller in reply to Aneliv9]

I am 68 and no other conditions and currently virtually no symptoms so taking 7 x 500mg hydroxy a fortnight and daily asprin and that is it. Don’t fancy a bmb so hope I remain ok or they find a cure - but just racing’s around doing everything I want to and enjoying life and most of the time forget I even have it - lots of yoga, gym and relaxation.

[Aneliv9 in reply to Timjonze]

I am just like your case, spleen enlargement 15cm, bone marrow MF1 and platelets 470. My heam diagnosted me as ET. The same was mentioned in my BMB results. Did your heam told you what your pace of progression looks like? Also what chance of progression gave you?

[Timjonze in reply to Aneliv9]

Hi Aneliv9, I think we are a rare breed indeed! The thing about all MPNs is nobody can tell you the pace of progression with certainty as it’s not easy to predict but in my case they’re quietly confident that it won’t be too speedy, especially as nothing has changed in last 18 months (had two BMBs and spleen measures to check progression) and my genetic testing didn’t reveal any awkward mutations. But everyone’s individual case is different. Where are you being seen? Do you have an MPN expert on your case?

[Aneliv9 in reply to Timjonze]

I live in europe, i dont think my heam is mpn expert, but she seems to know how to manage me up to now. I dont have the 3 known mutation either. I had my first diagnosis and BMB 3 months ago. So i dont know the pace of progression at all.. but MF1 makes me feel scared.. What your heam believes about a possible scenario that someone progresses to MF? Is he optimistic?

[Timjonze in reply to Aneliv9]

Hi yes they’re optimistic. Says I have the same chance as anyone else with ET which is roughly 10% over ten years and 20% over 20. With new drugs emerging I think the outlook is positive for us. I can’t really comment on your case but I was told that a small amount of marrow scarring was sometimes seen in ET and it doesn’t mean you’re necessarily getting MF ... it can be a very long journey from one to the other.

[Aneliv9 in reply to Timjonze]

Ok that about the scarring is very good,because i saw the diagnosis paper write: MF1 just after i arrived home. So i googled(!) and scared cause i thought that it is very rare. Your heam is also optimistic about the future of treaments of myelofibrosis?

[Timjonze in reply to Aneliv9]

To be honest we haven’t talked about MF recently but people in the know on this forum seem to be quite positive in terms of where we might be in 10 years time

[Aneliv9 in reply to Timjonze]

Thank you very much for your time spending answer to my questions.

[Timjonze in reply to Aneliv9]

No problem and good luck with everything!

[Aneliv9 in reply to Timjonze]

You too

[Aneliv9 in reply to Timjonze]

One more question.. You mentioned that you had mutation test and came back negative for all of them. Did you have some special test, or Jak,Calr and Mpl?

[Timjonze in reply to Aneliv9]

I have the Jak2 mutation but in terms of other risk factors for progression (eg ASXL1) they said there was nothing to worry about

[TM76]

I do not have Pre-MF, but my husband was recently diagnosed.

He is only 51.

We had no idea, but he lost a dramatic amount of weight and became quite fatigued. His spleen is at 32cm and causes him a lot of grief. I’m not sure of the grade of BMB, but will definitely ask!

I’m trying to research and understand as much as possible but am having so little luck. Even his Haem had no take home information about it for us, only PMF.

He is taking aspirin, a targeted chemotherapy pill and starts weekly phlebotomy tomorrow.

[kiwitraveller in reply to TM76]

There is not a lot out there on Prefibrotic - I went to a conference and the specialist told me I may or may not progress on to full Myelofibrosis- they just don’t know as this is relatively new. Currently my spleen is fine and I feel fine but my platelets fluctuate.

Is he on hydroxycarbomide - it is pretty standard - I don’t have any side effects but it does vary and they will alter dose to manage it. I have been on it for a year and still have to have it tweaked.

Hope all goes well. Where are you based.

[TM76]

Thanks for responding.

We are in Vancouver, BC Canada.

He’s taking hydroxyurea.

The main side effects / symptoms he’s experiencing are fatigue, enlarged spleen and weight loss. Apparently this medication takes longer to work.

That’s the underlying fear, I suppose, the not knowing and lack of resources and information.

Take care! I’m glad to hear you’ve been relatively side effect free!

[kiwitraveller in reply to TM76]

I hope the medication works well for him. It does seem to affect everyone differently but have met many people on it who tolerate it very well. All the best

[TM76]

Thanks so much! And to you as well.

[JeniMac]

I have just been diagnosed with this. I have had ET for 14 years