What is the rate for someone with ET to progress to myelofibrosis?

[Aneliv9]

I have slight increase in platelets and mild splenomegaly. My hematologist gave me 15% rate for progression to ET. Does anyone else has a percentage given from their hematologist?

[ccsial]

The rate increases with age and length of time you have had ET according to what I have read. I had ET for 15 years and at 74 now I have MF.

Mayo clinic stated the average life expectancy of ET is 12 to 15 years. There are people who have had it for a lot longer though. It really seems unpredictable.

[WileyFrench in reply to ccsial]

Actually what I’ve understood from this forum and from the literature is that people with ET can expect very near to normal life expectancy, assuming no complications from thrombosis etc.

[Hidden in reply to WileyFrench]

Exactly what I have been told from my Hematologist here in NZ WileyFrench.

[Richinspirit in reply to Hidden]

Ditto I agree.

[Anag in reply to WileyFrench]

Depends on age of onset. The younger it starts, the longer the life expectancy with the disease

[WileyFrench in reply to Anag]

Well, that makes sense. The older we are, the shorter our life expectancy is in general, ha ha!

[Anag in reply to WileyFrench]

😁

[katiewalsh]

Hi. That’s a good question & one I asked years ago. Everything I’ve read & been told since is consistent with what WileyFrench said. I think people get confused because many if not most of us are diagnosed so late in life that our time from diagnosis to death seems short because we’re already pretty old when diagnosed. So the cause of death is more likely to be a normal cause of death & unrelated to your ET. Some folks on here have had ET for 30 years and more. There are some gene mutations I believe which put some of us at higher risk than others but I understand that jak2+ is NOT one of the high risk mutations. Perhaps that’s what Mayo was talking about in a specific patient. Most hematologists seem not know anywhere near enough about MPNS to be relied on. My hematologist recently told me my fatigue couldn’t be from my MPN. But my MPN specialist said it’s quite likely. There is an excellent resource on the NCCN website for MPNs by the way. I know it’s hard but try not to worry & just enjoy your life as much as you can. We could all be hit by a bus tomorrow. Katie

[Aneliv9 in reply to katiewalsh]

Thank you very !uch for the answer,as all of you. I tend to worry TOO much all my adult life. I am 35 with 2 babies and my spleen size is what scares me the most.. also i may have the disease for 10+years... So i dont know in what levels my ET is. Sorry for my english,i live in Europe

[katiewalsh in reply to Aneliv9]

Your English is very good. Katie

[clubdino]

CALR positive people are more likely to transform to MF. I read an article that stated 25% of people that are CALR positive eventually transform to myelofibrosis. I've had CALR positive ET for at least 15 years and haven't made that transformation yet that I know of.

[Stanley28 in reply to clubdino]

Hi. Do you have a link to the article? It’s the opposite of my understanding so I would like to check it out. Thank you

[clubdino in reply to Stanley28]

I read that article over a year ago so I'm not sure where to find it. But after a quick google search I found a similar paper. As a warning, it's complicated to read. Here is an exact quote I lifted from the paper:

"Patients with ET who had CALR mutations had slightly higher ratios of progression to post-ET myelofibrosis than did patients with JAK2 mutations, but this difference was not statistically significant (21.4% vs 14.0%, P = .499)."

So that paper states 21% of us will transform to myelofibrosis. Here is the link to the paper:

academic.oup.com/ajcp/artic...

[WileyFrench in reply to clubdino]

I think that it says ET with CALR have the 21.4%, not patients with ET but without that mutation, so not all ET patients.

[clubdino in reply to WileyFrench]

Right. That's what I said in the post above that one.

[WileyFrench in reply to clubdino]

Ah yes- got it now.

[ChelseaF]

My doctors told me 15% also. I’ve done a lot of reading trying to get a clearer picture and it does seem that the length of time with the disease changes things. Also, the newer WHO guidelines may help with fewer misdiagnoses of people who actually have prefibrotic PMF as having ET. There may be some incorrect stats from earlier years when folks were diagnosed with ET and thought to have transformed but actually had MF all the while.

So basically, the more I read, the more I shrug.