Worried about this info

I have E.T and Jak2+. I had my 3 monthly routine bloods in March and saw a registrar for my results and consultation. I have read and been told that very rarely, E.T can progress to something much nastier later on. The registrar said that being Jak2+ i was at risk of this, and people who are Jak2 Negative do not have this risk at all.

I have been quietly mulling this over and getting more and more worried so thought i would ask on here if anyone else has been told this.

My platelet count had come down by around 20 from the previous tests. I know thats not much but i was pleasantly surprised. The little bit of joy that i had had the shine taken off by this information though.

Is this the case, that being + carries the risk, yet being - does not have the risk of transforming into something much more serious?

Sorry for rambling, ive been holding this in for weeks.

17 Replies

  • Hi Jeaniej1,  as far as I am aware any of the MPNS can progress to types of leukaemia but the risk is very small.  I have PV and jak2- but was told mine could progress also.

    I don't know exactly what the odds are but I don't think it's healthy to worry about something which may never happen.  The MPN Voice website will give you trustworthy information if you haven't already looked there.  I know it is difficult not to worry as I went through this stage myself but made up my mind if it does happen I will deal with it then.   I work in road safety and I think I am more likely to be injured on the road then my PV progress according to the stats!  Some of the other forum members will answer you I am sure with their ways of dealing with these worries.

    Keep in touch with this forum so you can get support from people who understand exactly what you are going through.  Kindest regards Aime x

    PS if you email Maz our editor direct at MPN Voice, she will help also 😺😺

  • Thanks for your reply Aime. I do look on this forum for my information and Maz has been great in sending me leaflets about these disorders and the medication used to treat them.

    I have managed to be positive so far, its just when i start to become comfortable with my diagnosis and not think about it all the time, that this shook me up.

    This forum is very supportive, and though i dont post much, i read new posts every day.

    Thanks again. Its so good to not feel alone.

  • Hi jeaniej1, you have put it in a nutshell.  We all, I think, accept and get used to our Mpn and jog along day to day until it throws a wobbler with something different happening and that's what causes the anxiety.  Andy raise a very good point though - there's a lot of people on the website have had their conditions for many years and are still here.

    My PV is throwing a wobbler just now - after having only 300 ml of blood taken off a few weeks ago cos I had a temperature, I now need more taken off despite having just gone through shoulder surgery.  There was a bit of a flap when I presented with a temp and had to get spleen and lymph nodes checked out but all was ok.  Just not looking forward to another two days wiped out after a venesection so soon again!  But after that I will be bouncing along again but means I can't have grandson this weekend coming because I'll be too tired but I'll have him another time.

    Keep in touch, kindest regards Aime x😺😺😺

  • Hi Jeaniej1,

    As Aime has already said the proportion of ET cases that progress to something more serious is very low and we all share that slight risk. I've seen suggestions that some mutations may be more prone to progression than others but given that the mutations are only quite recently discovered themselves and study sizes are small I doubt if it's as black and white as seems to have been suggested to you. Worth doing some research on MPN Voice and other reliable sites then discussing more fully with your doctor at your next visit. 13 years on from diagnosis I've learned that ET isn't nearly as scary as it first seems..!

  • Hi

    95% Of PV Patients are JAK2+, and about 50% of ET and MF patients. I have never heard that you are more likely to progress to MF from ET  if you are JAK2+.

    If someone is telling people that you are more likely to progress if your JAK2+ and have no chance if you arent, that sounds like guesswork to me and not the sort of thing he or she should be saying.

    As others have said the chances of progression are low, you are far more likely not to progress than progress.


  • I now see a Registrar. I'm not sure she knows anything about MPNs. Last time I was in and out in eight minutes (I timed it). I was 15 minutes in the pharmacy waiting room and I had a bit of good craic with the chap next to me. Much more fun than my consultation.

  • There is no difference in the rate of progression from ET to MF between jak+ or jak-. This rate, according to recent italian studies, is around 1/2% after 10 years from the diagnosis and 10% after 15 years for people who are over 60 years old and slighty lower for younger patients. Greetings from Italy :)

  • Look at the US forum for MPN 's narrated by Daniel Schorr. I believe I read recently that JAK2 positive is less apt to progress.  Interesting if I'm remembering right as that would contradict the Italian study.  Sometimes I think it's all just a crap shoot but that's my sarcasm. Try not to worry if possible; stresses is not good for your physical. & mental health. Katie

  • After every consultation I always read something different into the results, especially in the dark of the night and am amazed that I wake up in the morning 😳😱 My last few appointments have shown my platelets rising, so my HU dosage has been increased, so hopefully when I go to the hospital on Wednesday they will have fallen a bit.

    I have ET (jak2 neg) and was told when first diagnosed a few years ago there was a chance of transformation to something worse but I get so much "relief " when I read the posts on this site about others who do have "something worse" who are still with us after many years.

    We all do have something scary but we could all have something even worse or even get run over by a bus, probably the same odds of any of us winning the lottery 😄

    Best wishes 


  • Hi Jeaniej  , you've been given good advice by everyone, try not to worry about it , 

    Lizzie is right we could all have a much worse condition, I alway try to stay Possitive , it's much healthier for us ,

    I wish you luck on your next results 

    I also have ET Jak2 +  it's scary to be newly diagnosed I'm 3 years on now im in much better place, Thanks to MPN voice X

     I thank god every day after my TIA 3 years ago before Diagnose's it's only ET I have, 

    & I wasn't left with permanent damage, 

    Try to focus on the good things in Life 

    Don't worry about what if , enjoy life 

    You take care 

    Love Pam x

  • Hi Jeaniej, Phelpsy is so right....absolutely identify with her, as have ET jak + plus a former couple of TIA's...but have discovered that, 3 years on, ET is really very minor (esp in comparison with those with terribly serious conditions)...it is just so scary when you are first diagnosed and no one really understands (friends and family), just because it is rare. As life goes on, it just becomes 'part of the scenery' and a lot of symptoms we tend to associate with ET are probably nothing to do with it, either....it can be normal to get exhausted and to get headaches!! Now, when people with anxious faces ask me worriedly 'How are you?' I beam at them and say 'Absolutely fine, thank you' and promptly change the subject to something really positive (like the sun shining). It works a treat, as they relax, I relax, and immediately feel better! I do recommend it! But, of course, I do remember that when first diagnosed it was scary and this Forum just transformed everything and hope it will for you. Very best wishes. Keep your chin up! Tinkerbell13

  • As I have said before on this forum, please do not spend time worrying about what might happen in the future because all that can do is spoil your todays!  Statistics can prove anything and all analysis of potential progression in our diseases is worked out on historical information. The Jak2 mutation was only discovered just over 10 years ago and since then research in our field has really taken off and new medications are appearing which could completely change the picture. These are drugs that are being developed NOW so by the time it comes to your statistical low risk time for potential progression there might even be a cure. Get excited about that in place of worrying. The definition of fear is False Expectation Appearing Real. Think about that one and just enjoy life to the full day by day.

    Very best wishes, Jan

  • I agree totally with every one else,I am PV Jak 2,for six years now,the first months are dreadful and scary,I hate taking masses of Meds,I too was lucky in my diagnosis,was about to shrug my mortal coil,I was told afterwards,so have a stent and heart Meds as well as Hydrea.Now at the stage ,I know my French Consultant well,the nurse who takes my blood for testing here n my home every month,my G P,all great,I worry less now,am 76 yrs,still work as sculptor,there are tired days ,yes,but we must learn to stop when the brick wall looms!!!Very Best Wishes,you will be fine honestly,Jeannie.

  • Oh you are all such lovely supportive people! Thankyou  all so much for your common sense replies and putting my little "wobble" in perspective. You have all put my mind to rest again, more so than friends, family or the haematologists that i see. Thats because you are all living with this, or one of the other blood disorders in our exclusive club,  and can give advice from that position. Not what you learned in med school. Thats why the advice given here is so valuable, its first hand and real.

    Thank you all again for taking the time to reply to my post, i feel so much better.

    Good Health to you all

    Jeanie. X

  • Hello JeanieJ, I am sorry that you have been feeling so confused by the information you were given by the registrar, I have asked Prof Harrison for clarification on this for you, she has said:

    This info is not really true at all, all ET patients have risk of transformation and there are many facets that are taken into account when this is evaluated. I suggest they contact the department again...

    I hope this helps.  Best wishes, Maz 

  • Hi Mazcd

    Thanks for clarifying this for me. I will probably see someone different in June when i next go for blood tests, and i will mention the information given to me by the registrar and also from Prof Harrison.

    On a positive note, Cambridge University are doing research into MPN's, and on my last visit to Haemotology  in Sheffield, I was asked if i would be willing to donate extra blood to be used in their research programme. They are trying to figure out why these disorders happen, what makes certain peoples genes mutate and ultimately find a cure. I jumped at the chance to help! My blood will be taken at the same time as routine blood tests. It is anonymous, but will have a "code" so that i can be tracable if they find anything that may need further investigation or treatment.

    Thanks again Maz for being so pro activley helpful.

    Jeanie. X

  • That' right, prof Harrison is right. All ET patients have risk of transformation. I read and translate for italian patients many studies every week and I know that currently there is no way to prevent progression or identify patients who are at higher risk.

    At the same time, the disease could be very different: in MF, the recent prognostic score, establishes different survival ranging from few years to more than twenty years! In secondary myelofibrosis italian and european hematologists are working on a different e more accurate model to predict survival and this is a good news because they are going to be able to discriminate between patients and treat us better.

    Again, survival in MF post TE or PV ranging from few years for people who are at higher risk to decades! So, my opinion is: celebrating life and live day by day and step by step. I'm 31, I'm looking for a new job (not a good time to live in Italy, unfortunately..), I want to marry my girlfriend so I CAN'T SPEND MY TIME THINKING ABOUT MY DISEASE.

    It seems that the key is not jak, calr, mpl, but the minor mutations: they drive the disease, many patients are stable for decades and they have jak, calr or mpl because they are not the target...in the world, many hems are going to discover that there are other and more aggressive mutations (SRSF2, TP53, ASXL1) which will be targeted in the next future.

    I'm very hopeful and excited about this...I'm sure that in a decade or more it will be possible to slow or arrest progression or, at least, to make fibrosis reversible and live many years healthy.

    Trust me ;) 

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