Had appointment with my MPN specialist yesterday and it was the shortest one since I was diagnosed five years ago. We reviewed my most recent blood work and had to look at it twice. - ALL readings were within normal limits…Platelets at 395 were under 400 for first time in likely almost 20 years..WBC right in middle all other bloods where they should be - liver and thyroid fine…
My MPN specialist was as pleased as I was..he reminded me that this is a marathon - it’s taken two years of treatment with Peg to get to today and a lot of ups and downs and adjustments in dosing; a break due to liver readings; going on thyroid meds but so far all worth it…my treatment goal has always been QOL over numbers but if Incan have both will take it!
He also congratulated me for pushing to go on Peg. I had initially started on HU but given my age <50 at the time and the fact that I was pretty convinced that we were/are dealing with pre- fibrotic PMF and not ET (due to very high Jak2 burden; persistent leukocytosis (high WBC); some minor fibrosis in the BMB and the fact that it took three specialists to try and determine if we were dealing with pre-fibrotic PMF or ET) - I wanted to try the only treatment for ET known today to perhaps stop progression…at the end started on HU but kept pushing and it became a moot point when I didn’t tolerate HU anyway (was HU intolerant more than resistant)…
For now we are going to continue at steady state (90 every 2 weeks). If things stay stable in next two appointments (he kept them at every 4 months as he didn’t want to stretch to every 6 months yet (and neither did I)..then we will re-test allele and perhaps take a look at bone marrow again.
Just wanted to share some good news as well relate that it can take some time and definitely not be a straight line (lots LOTS of ups and downs) but as my doctor reminded me - if we are lucky this is a marathon….
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Solyesh
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Someone just DMed me about potential difference between ET and pre-fibrotic PMF - I forgot to add that in addition to 1) very high (for ET) Jak2 allele burden; 2) persistent and high leukocytosis; 3) some reticulin fibrosis (although minor) - I also had extreme hypocellularity for my age (also usually not found in ET although present in pre-PMF and in PV but my RBCs outside of platelets have always been WNL).
The differential diagnosis between ET and pre-fibrotic PMF can be very difficult. I believe the WHO came out with new criteria in 2020 (right after my initial BMB in Dec 2019) which is why it took 3 specialists to try and determine if we were dealing with ET or Pre-PMF.
For me the only difference is I would have been even more aggressive on insisting on starting on interferons right away and not watch and wait or HU…
Solyesh that's fantastic news! Sounds like you and your doctor are on the same page. Do you mind sharing your allele burden # and if it was from serum or bmb?
Ok. Thanks. I thought I had read somewhere that allele burden was not a concern until it reached maybe 50% or even higher. So hard to understand everything I read and even harder when the experts don't always agree
That's all very interesting and very useful information to me for my current transition from hydroxy to peg, I am six months in, review tomorrow at hospital clinic. I have a Tet2 mutation and the allele burden is quite high (32%) and I believe they are not sure if peg is as effective at reducing the burden in Tet 2 as is the case in the more common mutations, time will tell. Good luck on your journey, it all sounds good.
Janis - Do you also have a Jak2 mutation as a driver? I too have TET2 but at 4% burden and the only discussion I’ve had with my MPN specialist around it is that the most recent research seems to indicate the order in which the mutations were acquired is important? Others on the forum have chimes in in past with much better information. Best of luck at the clinic!
Hi, no other mutations other than Tet2, I am not sure if that is good or bad and sometimes I feel that I do not want too much information just in case I worry myself to death. I was told that 10% of the elderly population have a Tet 2 mutation and that Tet2 is a driver of inflammation (probably the cause of my long term aches and pains). Getting my list of questions ready for tomorrow, I bet they dread it when they see the bit of paper coming out of my bag!!
Ha! That is great - I also always have my list of questions at the ready and very purposefully go through them one by one - if there are a lot of complicated some times I will forward to the doctor a few days in advance as a heads up about what's coming and my MPN specialist has said he appreciates that (not all the time but once in a while)..we have to be our own best advocates!
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