High Risk?

[lizzziep]

I’ve seen the consultant today, all the NSG results are now back. He said they showed I was definitely transforming to MF, also that some markers showed I was in the high risk group for transforming to Leukaemia. I found that very scary. He said it would probably be a few years but that I would be monitored. I’m 70 now.

Has anyone else been told this and if so what happened?

I’m now going to be on peg interferon, starting on 45. Got to see the specialist nurse to be shown how to reduce the dose from 90 to 45. Then should be able to do it myself. I’ve been doing EPO injections ok. Carrying on with one Anagrelide capsule per day until interferon kicks in.

My blood counts are good, only platelets (600) and RDW (17,5) out of range, everything else in normal range. The EPO injections have worked, I was very anaemic and am now in normal range - just.

I’ve been warned about possible side effects of the interferon.

I have a symptom checker booklet - know your score. Out of a possible maximum score of 100 mine is 10. I have hardly any of the symptoms - out of 10 symptoms listed, I have very low scores In fatigue, early satiety, inactivity, low concentration, night sweats, spleen pain (rarely), itching - occasionally on my hands. Bone pain, fever and weight loss (I wish), all negative.

I’ve also been recommended to have an eye test.

I’ve actually been feeling really well recently so the thought of leukaemia is quite a blow.

[mhos61]

Hi Lizziep.

I’m really sorry to hear of this news, and can totally understand how you must be feeling. I hope some of our very knowledgeable forum members can assist you with your questions.

I can only offer my thoughts to your situation.

I would definitely want to have access to the NGS panel results and have their significance thoroughly explained to me. For instance, what ‘markers’ have been identified to categorise you as ‘high risk’ for Leukemic transformation.

I recall you’re not Jak2+, but did they find a CalR or MPL driver mutation’ or confirm ‘triple negative’ status.

This might be a good time to get a second opinion from an MPN Specialist.

Best Wishes x

[lizzziep in reply to mhos61]

Thank you.

[Otterfield]

I'm really sorry you have had such frightening news. Remember, a risk doesn't mean it's going to happen but it's a very unsettling position to be in. Given that, you might want to discuss SCT with your consultant. If you are otherwise healthy, your age should not be a barrier - a member of this group has recently had one at 70. I don't suggest this lightly - as you may remember, mine was recent and clear in my memory - but I have no regrets.

[lizzziep in reply to Otterfield]

Thank you.

[UKZA]

hi there, sorry to hear this. I have post ET MF diagnosis at 53 with CALR, TET2 and IDH2 mutations putting me at an intermediate risk. I have recently met with the Transplant team and they have said that this will be considered up to 70 if I remain fit and healthy. Find out what your mutations are and discuss a SCT . Remember that the prognosis is a median ( mine was 3.5 to 6 years) but a strong fit person will outlast this. (So I have been told) I am on interferon now and it has some manageable side effects, but seems to be doing it’s job. I have been told I can move to ruxolitinib if MF burden becomes worse. I hope you find some answers. Take care. Penny

[lizzziep in reply to UKZA]

Thank you.

[katiewalsh]

Hi Lizzie, is your consultant an MPN specialist? If not, I strongly encourage you to see one who is. I was diagnosed with MF 10 years ago. I know I’m jak2+ but no one has ever talked to me about the other things. I’m 72 with other health issues so maybe they don’t tell me because I wouldn’t be a candidate for SCT. I’m really sorry you received this upsetting news. I was told in 2013 I might only live 8 years & here I am with decent blood counts & only fatigue as my symptom. When I finally met with an MPN specialist I received a very different prognosis. Please know I’m sending you positive thoughts for dealing with this news and situation. And e-hugs. Katie

[lizzziep in reply to katiewalsh]

Thank you. He did say he had consulted another team, but I don’t know who! When I go for the injection ‘lesson’ I will ask if I can get the full NSG results.

[Shelly1960]

Hi sweet although a scary time ask questions we can deal with what we know and much love

[Xuzy]

Hi lizzziep

Did you get a bmb?? Because that's the only way to tell if you are headed for transforming into myelofibrosis.

[lizzziep in reply to Xuzy]

Yes, it was the bmb samples that were sent for NSG testing and the results of that showed transformation.

[Mtnlife]

Sending positive thoughts and prayers your way! I know this is scary. You seem to be doing all the right things!Beth

[ainslie]

well, I am not a expert but my instincts are don’t jump the gun just yet. I have never heard of someone been told that from NGS, proper diagnosis is usually from a Bonemarrow biopsy as far as I am aware. my understanding of NGS is it tells you what mutations you have , I was not aware it can even tell you you are transforming to MF, I’m very sceptical about that. Some believe some mutations may possibly have worse outcome but I think it’s a very grey area and as Otterfield said having a mutation indicating a POSSIBLE higher risk doesn’t mean it’s going to happen. Eg there are plenty people out there with Jak2 that don’t have MPN and may never have it. I am surprised your doc didn’t do a biopsy in view of his concerns about MF and leukaemia risks. I think that’s concerning. If it were me I would ask as somebody else said for your doc to explain in detail his diagnosis theories from only a NGS and ask also for a biopsy. If you have given us the whole picture I think your doctor may not be optimal. Personally I would get a second opinion from a MPN expert and show them the NGS labs and the slides and report from the biopsy. In the meantime keep positive until you get the full picture and hopefully from a expert.

[lizzziep in reply to ainslie]

I had a bmb and it was samples from that which show transformation via the NSG tests.

[ainslie in reply to ainslie]

ah, I don’t think you mentioned Biopsy in your original post, NGS can also be done just from blood, although I hear it’s better from marrow perhaps. Apart from now knowing you had a biopsy, I think the rest of what I wrote is still worth considering, including the part re a risk doesn’t mean it’s going to happen. There is a school of thought that ET is only definitely ET if confirmed by a biopsy, some say ET is SOMETIMES undiagnosed MF., it can be a fine line. As you know MF can vary in how serious it is. From what you say it doesn’t sound so bad now. I know some veterans with MF still doing well after 20 and 23 years after PV and ET. Whether it’s a coincidence or not they are both on interferons, so maybe good your on it now. Also drugs are changing now , even today there are several for MF and many on the way. I always think a second opinion from a MPN expert is a good idea, can’t do any harm apart from upsetting current doc if ego issues. The good thing is you feel well now, do all you can to get the best doc and treatment you can get and make sure to enjoy life. I always regret how much valuable time I wasted worrying unnecessarily about my PV after diagnosis, I know it’s hard but we have to try. Keep us updated on how it’s going.

[lizzziep in reply to ainslie]

Thank you. I have decided to try and get an MPN expert, preferably Prof Harrison’s views on my case.

[ainslie in reply to lizzziep]

thats a fantastic idea, she would be perfect

[Cassandra61]

Sounds like you have had a really tough time. Hope you are looked after well and keep in touch and let us know you get on

[saltmarsh]

Lizziep - I can't add anything to what's been posted except my best wishes. These "wrinkles" on our journey are certainly unsettling but it sounds like you're moving in the right direction.

Glad you're feeling well and asking questions. Keep moving forward.

[Cja1956]

Hi, Lizzziep, so sorry to hear about your recent progression. I also progressed to MF from ET about four years ago. I was pretty stable for a couple of years, but came very symptomatic about a year ago and now I am in consultation for a stem cell transplant. I don’t have any markers for leukemia, but if I did, I would definitely talk to a mpn specialist regarding a SCT. I wouldn’t really trust your own symptom score list. The doctors know the right scoring tests to give us. I also mention the SCT because it sounds like you’re pretty healthy right now and you don’t want to wait until you’re too sick to have it. You also might be rapidly approaching the age where you couldn’t have it.

I wish you all the best. Please keep us us up to date.

[lizzziep in reply to Cja1956]

Thank you, I’ve decided to try and contact Prof Harrison for advice, I’ve put a post on asking how to go about it. The score I mentioned is from the booklet they gave me at haematology to note different symptoms on different days. Hope your sct goes well.

[Cja1956 in reply to lizzziep]

I understand. I just wanted to give you my take on it.

[Otterfield in reply to lizzziep]

Your haematologist can refer you to Prof Harrison, that's what mine did. I still have telephone appointments with the team at Guy's every four months, so they are also monitoring my recovery.