Should I or should I not?

[Kelly2]

Hi there. I finally got results from my BMB. Although I had another one last September and they both don't say anything about progression or myelofibrosis. On the contrary I found out that it says I have Essential thrombocytosis which is different from polysytaemia vera I thought I had. Right? The thing is the junior doctor with whom I talk says the BMB shows no progression but my counts show signs of myelofibrosis and they want me to start Jakafi!! I am terribly perplexed and I would like your advice on this. I hoped for Besremi. Should I insist? I have an appointment on Monday with the Registrar who told me on our first meeting that I am in denial!! I am very upset. Please help.

[lizzziep]

I’m not as knowledgeable as others on here but I thought the BMB should show whether you had MF. It was that that confirmed my progression from ET to MF. I was getting more anaemic. I was on hydroxy then Anagrelide for the ET. I’m sure someone else will give more information to advise you. Hope all goes well whatever medication you end up with.

[Kelly2 in reply to lizzziep]

Thank you. I am so mixed up with this. The BMB says no MF is observed. I will have a second opinion on this.

[Kelly2 in reply to lizzziep]

Thank you. All the best to you too!

[ainslie]

you could ask the junior doc to explain what in your counts imply MF, do you know what your counts are?, better you need to see a expert Haem to explain clearly which MPN you have. From what you have said I can’t see how you had ET or PV with no progression and you now allegedly have MF, it doesn’t make sense so far.

[Kelly2 in reply to ainslie]

I will try to have an appointment on Friday with a specialist to show her my BMB and blood tests before I meet with the doctors at the hospital. I hope that she will put things right for me. I need a second opinion before taking any medicines. Thank you. I will keep you posted.

[ainslie in reply to Kelly2]

i think thats a great idea, BMB are really best interpreted by a MPN expert

[Kelly2 in reply to ainslie]

Unfortunately she had no appointments for me. So I am on my own on this. I'll see what explanations he will give me on Monday.

[ainslie in reply to Kelly2]

Sorry to hear that, I think it would be useful if you could arrange an appointment with a MPN expert. We are not experts on here but you may wish to post your CBC

labs on here.

[hunter5582]

You need to consult with a MPN Specialist about your BMB results. This is particularly true when there are ambiguous results.

There are differences in the bone marrow morphology when distinguishing ET from PV and both from MF. There are additional differences in other symptoms as well. pmc.ncbi.nlm.nih.gov/articl... Here are the differences in bone marrow morphology.

Polycythemia Vera - BM biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size).

Essential Thrombocythemia - BM biopsy showing proliferation mainly of the megakaryocyte lineage with increased numbers of enlarged, mature megakaryocytes with hyperlobulated nuclei. No significant left-shift of neutrophil granulopoiesis or erythropoiesis and very rarely minor (grade 1) increase in reticulin fibers.

Prefibrotic Myelofibrosis - Megakaryocytic proliferation and atypiab, without reticulin fibrosis > grade 1c, accompanied by increased age-adjusted BM cellularity, granulocytic proliferation and often decreased erythropoiesis.

Overt Myelofibrosis - Megakaryocyte proliferation and atypia accompanied by either reticulin and/or collagen fibrosis (grade 2 or 3).

Note that it is not uncommon for people with ET/PV to have a low level of fibrosis present. The longer the condition is present, the more likely some level of fibrosis will be present. It is a concern when fibrosis is present, but it does not change the diagnosis to MF. It indicates the possibility but not the certainty of progression into MF. The presence of detectable fibrosis does speak to the need for treatment with the most appropriate medication, one that reduces risk of progression.

Besremi and Jakafi are both viable options in your situation. For what it is worth, I would consider both. Choosing between these two would depend on your MPN profile, your individual treatment goals, and your risk tolerance. Making the decision requires careful consideration of both options. This is a discussion best made in consultation with a MPN Specialist. It is also a decision you need to make in consultation with a doctor who you trust, respect, and who respects you and your ability to make decisions.

It is your prerogative to seek a second opinion before making a decision. I would not make any significant treatment decision about anything without a second opinion. You need and deserve to feel confident that you are making the right decision.

Wishing you all the best moving forward.

[Kelly2 in reply to hunter5582]

Thank you Hunter. You are always reassuring me! I am waiting to make a phone call and hopefully I will be able to see the professor tomorrow. Before I see the other doctors . She is responsible for the transplants at the hospital and she is my last chance for reassurance! Besremi is more appealing to me in the sense that you don't have to take pills everyday! I will keep you posted.

P.S

Unfortunately the professor was not available even in her private office for a long time. My bad luck continues. So I will have to discuss with that one doctor. I will let him know of my fears and goals and I'll see what happens. I am without therapy at the moment and tomorrow I will have blood tests. I really envy you with the health care system and the professionalism of your doctors there! Here the patient is supposed to listen and not speak and this is not my case...

[Richinspirit in reply to Kelly2]

What country are you in Kelly2?

[Joetcalr]

my understanding is that you get put on Jakafi if your spleen is enlarged, and on hydroxy or peg if it isn’t.

[Kelly2 in reply to Joetcalr]

Mine is enlarged.

[Loubprv Volunteer]

Good grief You poor thing. Where are you?

It would be so helpful if this website highlighted where we all live and I can't understand why it doesn't.

Junior doctors are not always au fait with mpns in my experience - unless they are being specially trained in these particular cancers.

May I suggest you jump ship and seek a second opinion ?

To say you're in denial is in my book, very bad practice and simply not on.

I m 70, on Rux ( Jakafi) and have pv - diagnosed 2009.

Excellent drug so far. No enlarged spleen by the way but I do have a superb haematologist in Manchester.

Interferon didn't suit me at all - had horrendous side effects, so I wasn't offered Besremi.

Hope you get the right diagnosis and treatment very soon.

Louise

[LFCLove in reply to Loubprv]

Hi Louise, when you set up your profile, it is your choice whether to allow people to see where you are in the world or hide it.

[Loubprv Volunteer in reply to LFCLove]

aha didn't didn't realise this, silly me.

However, no one seems to use this facility.

Seems to me it would be so much easier if we had to Inc inour location.....

[Kelly2 in reply to Loubprv]

I am in Europe and better anonymously. It's a good thing Jakafi works for you. Maybe I shouldn't be so negative about it! I can always try it and see what happens. I would prefer Besremi because it is not an every day drug. But I can always try Jakafi since they insist. Thank you.

[DiveGoddess]

Kelly2,

I send you positive vibes and prayers to deal with this difficult situation.

I am sorry your Doctor has not given you more clarity. Anyway you can talk with Head Administrators and request consult with Senior personnel or Specialist?

Your concerns are valid. I am hopeful you will reach someone who can explain better and discuss why a certain treatment modality would be better for you.

Please keep us updated. Please know your Healthlocked community cares!

[Kelly2 in reply to DiveGoddess]

That's what I plan on doing on Monday. Seek explanation for his opinion. If I ask for somebody else I will be put in the black list and I don't want that as I had trouble in finding and being accepted in this hospital which is specialising in MPNs. I am calmer now and we'll see how it goes. Thank you.

[Spanelmad]

Would highly recommend asking for a referral to a specialist for a second opinion.you can then set up shared care.Maz will have the up to date mpn consultant lists.

[Kelly2 in reply to Spanelmad]

I do not live in England and very few hospitals specialise in MPNs. I am lucky to have found and been accepted by this one. I will make the best of it and listen to what they have to say. Can't do anything else anyway. Thank you.

[Br0wng1rl]

Hello Kally, I am PV patient and taking Jakafi for the last two years. When I got done my BMB at my local hospital two years ago, the diagnosis was that I have ET. However, my MPN specialist in New york had the bloods slides go through his labs again and I was told that I have masked PV. At that time, my platelets were really high like over a million and red blood cell were slightly high.

I would advise you to go see an MPN specialist for this issue. I hope this helps and Good luck with the process.