I'm new here and just trying to learn as much as I can about MPNs. I was recently diagnosed with Jak2+ ET, however the pathologist who completed my BMB indicated that there are findings not typical of ET and he can't rule out pre-fibrotic PMF. My hematologist indicated the treatment would be the same at this point and she will proceed as if this is a positive ET diagnosis.
I was completely caught off guard when I was given my diagnosis, so didn't have any valuable questions to ask at the time. After much research, I'm learning that while the treatment for ET and pre-fibrotic PMF are often the same, the prognosis can be much different.
Has anyone else had a BMB where the pathologist wouldn't make a clear diagnosis? Is it worth trying to get a 2nd opinion for the BMB? I don't have any reason to not trust my new hematologist; she's only working with the information she's been given and acting accordingly, but it doesn't sit well with me that we don't have a clear confirmation of which MPN I'm actually dealing with.
Just curious if anyone else has been in the same situation and if they were able to get a clear diagnosis with a 2nd opinion, or if this is just a "wait and see" scenario.
Thanks! 😊
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WRLM
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This does happen. There can be a number of reasons why the answer to your BMB results is not entirely clear. It is definitely advisable to get a second opinion. This has nothing to do with trusting your new hematologist. Getting a second opinion on your treatment is simply a prudent thing to do with any significant medical diagnosis and treatment decision.
MPNs are rare disorders. Most doctors, including hematologists, have little experience with MPNs. Consulting with a MPN Specialist for a second pinion or for your ongoing care is essential in managing any MPN. No competent ethical doctor would mind you seeking a second opinion. They would encourage it.
Here is a list of patient recommended MPN expert doctors. mpnforum.com/list-hem./ Suggest it is worth doing whatever it takes to consult with one. A MPN Specialist can best advise you as to your diagnosis and treatment options.
Thank you so much for the info! I'll try to locate an MPN Specialist in my area. My hematologist is now on a 3-month sabbatical, so it would be nice to have someone else to field some questions in the meantime. I really appreciate the advice. There's so much info to navigate in the beginning it's a bit overwhelming. 🤯
In my case the marrow pathologist said "suggests ET" but added that other clinical info (outside of his lab) should be considered. My Dx is PV. It could be "suggests" and the like is common in these reports since so much is vague.
Prefibrotic PMF is a relatively new class officially separated only upon the 2016 WHO criteria.
This report (getting old at 2017) has some ideas on the Dx of pre-F-PMF. wherein they note the "existence of pre-PMF has been questioned". They offer a complex formula to predict it, a sample of efforts to address this uncertain Dx.
I had a BMB and the first said Pre-MF and had even graded the fibrosis after I had been diagnosed with ET, like you I was completely thrown by both so had nothing to ask. I was them referred to a specialist and their pathologist who is much much more familiar with MPNs looked at the biopsy and said there was no fibrosis and said consistent with ET. So at risk of sounding like a broken record a really recommend a second opinion from an MPN specialist. It sounds like your current dr is good, but I have learned; and the article in The guardian shows non specialists are lacking experience and knowledge that can lead to drastically different diagnoses
as others mentioned , time for a second opinion from a expert, it’s best if you can give the slides in addition to the report to the second opinion doc. Sometimes docs see different things when looking at the slides. Leaving you with the view it could be ET or MF is pretty poor, I would want clarification or at least a second opinion.
New MPN doc I just saw suggests MPN as an umbrella diaganosis is gaining ground because of the overlap between ET PV and MF and that treatment options are often similar.
My BMB experience includes two biopsies. First 8 years ago pathologist said ET with 0 fibrosis out of 3. Many docs over the years agreed until last fall a new pathologist looked at same biopsy and said he saw Primary MF. The doc there said pathologists often disagree. New BMB a few weeks ago said findings consistent with ET with “no significant fibrosis”. Have not seen a grade on fibrosis yet.
Overall goal IMO is to be treated just enough to have good quality of life. Sometimes more meds is worse than the disease itself.
In my case, I had a high PLT count and JAK2 mutation, but my RBC and WBC counts were within the normal range, which ruled out other MPNs, but my BMB was inconclusive for ET by the new WHO standards back in 2017. However, I was 66 years old at the time and classified as 'high-risk', so my hemotologist/oncologist went with the older ET classification that didn't require a BMB (British, I think) and started me on Hydroxyurea right away, which I still believe was the right call.
I see by table 2 in the paper linked to by EPguy that a pre-PMF diagnosis must first rule out an ET diagnosis, so did you not have a high PLT count, WRLM ?
Thank you for responding. My platelet count has been slowly, but consistently increasing since 2016. I didn’t have a family dr at the time, but I’ve taken all the lab reports to 4 different walk-in drs over the years, and they all said “well, they’re not THAT high” and not a one was the least bit concerned.
I was finally able to get a new gp and without me even bringing it up she reached out after sending me for some blood work, saying my platelets were high and she noticed they’ve been high for awhile now. She asked some follow up questions (night sweats, headaches, fatigue etc) and when I said yes to all of those things she sent off a referral to a hematologist, saying it might be nothing but she wanted to be sure.
After tons more blood work & a BMB, here I am having been diagnosed just 2 weeks ago. I’m confident I’ve had this for years, so I’m very grateful for my new Dr following up.
All my other numbers are within the normal range. My RBC was juuuuust over one time, but then back to normal the next. So seems to only be a platelet issue at this point. Even at that, my count has never been higher than 600 yet. I was so surprised to see how high some other people’s numbers are. I do have lots of symptoms, so I can’t help but wonder if I’ll continue to feel worse as that platelet count gets higher.
I think the advice to get a different pair of eyes on those slides is sound and I’m going to figure out how to make that happen, just for a little extra piece of mind.
It seems I’m going to have to adapt to being ok with so many unknowns when it comes to this condition, which is definitely going to be a challenge for me. Maybe a concrete diagnosis isn’t as important as I think it is since this seems like more of a spectrum type situation.
I’m so happy to have found this group - I’m learning a lot from all these responses, and reading thru other people’s posts.
I also had night sweats, fatigue, headaches, etc., but I had no clue that my PLT count was higher than normal for who knows how long, because I was one of those people that rarely went to the doctor for anything until symptoms became unmanageable enough to not deal with on my own.
Similar to your experience, my PLT count never got above 550, but it was always well above 400 until I started taking Hydroxyurea. Taking 81 mg aspirin twice per day didn't do anything to reduce the PLT count ('watchful-waiting' prescription), but doing that is still import to help reduce the probability of having a thrombotic event.
The good news is, I started taking Hydroxyurea once per day and that helped to get my PLT count comfortably back into the normal range, but it doesn't stop there. I still get blood tests every three months so my hemotologist/oncologist can review the results to see if there are any markers that may indicate that a change in my treatment plan is warranted, which is comforting, because transformations can happen with MPNs.
I found the same, no effect from aspirin on PLT, But I did see a correlation to reduced HCT, it went down fast once I started aspirin. This is seen often in the literature, a sample:
"In two studies ... falls in Hb or Hct after baseline were reported in patients taking LDA (low dose aspirin) and in those taking placebo"
Hi! I had a a BMB last month and the pathologist report was very similar - with elements of both ET and pre-fibrotic MF. I posted about it on this site, so you can look up my posts for more details.
When I talked to my doctor (an MPN specialist), his assessment was that ET was likely the correct diagnosis, given the length of time that I have had an MPN and the fact that I did not have any fibrosis. He did indicate that there are overlaps between the MPNs, coupled with the fact that they can change/progress, so frequently there is complexity in the actual diagnosis. My doctor was more focused on managing symptoms and quality of life. For me, right now my symptoms are not significant (fatigue is the biggest), so continuing with aspirin and monitoring platelets (right now ~700).
I had asked also asked him if the BMB findings that fell in the pre-fibrotic MF category (which were high cellularity and high M:E ratio) were things that meant that (assuming the ET diagnosis is correct), that I had a "higher risk"/worse ET. He did not think so.
When I talked to him, it was telephonic (which is always a bit harder than in person), and of course as soon as we hung up, I had more questions! But I have been writing them down for my next appointment, which will be in September. Seems like a long way away, but at least at this point, I don't feel a huge sense of urgency related to my personal situation (i.e., nothing is going to cure my MPN at this point, symptoms are manageable, etc.). I do want to ask about the value of considering interferons - the only thing that I am aware of that may have some ability to slow progression. When I thought I was firmly in the "ET camp", I hadn't really thought about it too much, but now with a potential toe in the water of MF, it is something that I am curious about.
This is very helpful - thank you for taking the time to share. I’ll definitely try to find your other posts.
My first 2 appts with my hematologist (including telling me my diagnosis) were on the phone as well. Now that I have a diagnosis she wants to meet me face to face, which will happen in July.
I was so caught off guard by the diagnosis (had never heard of these disorders before) that I had zero questions after she told me. Now that it’s sinking in and I’ve done more research I have a long list of questions for her. Unfortunately she’s gone on a 3-month sabbatical, so it was poor timing for me.
This site has been really helpful and I’m learning a lot from everyone. It’s also generating more questions, which is good too.
It must be very difficult being newly diagnosed and have limited access to your doctor. Hang in there and try not to worry too much. Not easy particularly in the early days of diagnosis and not having answers to all your questions.
considering trying Bes or Peg might be helpful, it seems it potentially slows progression for it appears a small minority, worth discussing with an expert perhaps
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