BMB

[Bmj63]

Diagnosed with et mpl positive 18 months ago.consultant said she didn’t

Need to put me through an uncomfortable bone marrow biopsy.is this normal practice.

[Rem31]

I was diagnosed in 2013 and the only reason I had a BMB was because I had tested negative for the then known mutations and everything else considered at the time. The BMB was the only way to confirm my diagnosis and I understood it was standard diagnostic practice for my case.

Maybe others will say the practice has moved on but I wouldn’t expect to have an invasive procedure for no good reason.

[Otterfield]

Perhaps things have changed. However, if you do have one, it's not necessarily that bad. They use local anaesthetic and mainly you feel a lot of pushing and minimal pain. Others might have had a different experience though.

[clematis26 in reply to Otterfield]

Hello - how are you? Thinking of you and wishing you a Happy Easter!Take care, Sue

[Otterfield in reply to clematis26]

Happy Easter to you too. Feeling quite rough at the moment and the dreaded itching has returned! I will still be in hospital for Easter and beyond. Jennie

[Meatloaf9 in reply to Otterfield]

Wishing you all the best on this leg of your journey. Hang in there, you will come out better and stronger. Happy Easter to you.

[Threelions in reply to Otterfield]

Hi Jennie,Just a quick message of support from me. Hope you’re doing ok. You remain in my thoughts & prayers. X

[Cja1956 in reply to Otterfield]

Hi, Jennie, thinking of you, as well and hoping you start feeling better soon. Sending hugs.

[hunter5582]

I have had a MPN (ET that progressed to PV) for 30 years. I have never had a BMB. All of my docs, including 2 MPN Specialists, said I did not need one. Different docs have different approaches to BMBs. Some do them routinely on new MPN cases. Other do not.

My own plan, in consultation with my MPN care team, is to do a BMB when there is a reason to do one. This would be when there is some sign of MPN progression.

Suggest you decide for yourself whether the benefits of knowing your exact bone marrow status outweigh the discomfort of the procedure and the minimal risks involved. It really is just a judgement call.

[Paul_1971]

I had one but at the time because of varying reasons it was unsure if i had PV or MF.

However i understand if blood counts, mutations etc point to a condition that would be considered enough for diagnosis.

[mjfin72]

Hi I also have et mpl. Diagnosed in 2016 and have never had to have a bone marrow biopsy. Best wishes

Mandy

[LT55]

I was diagnosed in 2015 and hematologist (not an MPN expert) did not suggest a BMB. Over the years I heard that a BMB was important, but honestly, a BMB just sounded awful, so I was just not going to be the one to push it. I recently saw an MPN expert asked him about a BMB. He told me that give the 7+ years of blood work (with bloodwork 2x-4x per year), he didn't see the value in it unless I had an interest in knowing if there was fibrosis. With or without fibrosis at this point, he would not change the treatment.

So for my personal situation, it was really more about whether or not I wanted to understand if things were progressing. My decision thus far has been: "No, I'm good being in the dark." Even if no fibrosis now, no guarantee it would remain this way. And if there was fibrosis, and since treatment would not change, I really don't want to know that things will be progressing/worse in the future - would just make me worry.

Given his response was based (at least to some degree) on the long history of bloodwork, I wonder if he would have recommended a BMB initially when there was not that history. I have switched to him as my primary treating MD, so perhaps I will ask him at my next visit.

Best of luck to you.

[EPguy]

I had BMB near the time of my Dx. I'm ok it happened, but it was not comfortable. Knowing fibrosis condition may have future value if or as treatments are able to have an effect on it. For example some studies show interferon (INF) can reduce fibrosis in some cases (topic of some threads). If this proves true, having an early reference point will be good. Other discoveries from BMB that arise may also be relevant for future therapies.

But all this is more theory at this point, so BMB for this is more "in case it's useful later".

Another minor finding, my BMB had an allele burden 5% higher than the blood panel showed. They usually are similar from studies we've discussed on the Voice, but my case shows there can be some differences.

In a related item, if you have access to a full genetic panel (NexGen sequencing) this also may find (or not) things that prove to be useful info for future therapies.

[LT55 in reply to EPguy]

Great perspective. Something for me to think about. Thank you.

[Pte82]

Bmj63 is your consultant a MPN specialist?

[Wyebird]

Hi and welcome, Heamo in my understanding is absolutely right. A mutated gene has been found.

BMB is usually when heamo detects a possible change in your bloods and wants clarification. Sometimes no mutations are detected hence a BMB.

[dabs121]

Hi Mmj63, I had the same diagnosis as you at the end of last year and haven't had a BMB either. My haematologist also said it wasn't necessary to put me through it as it wouldn't change my diagnosis or treatment (HU & aspirin). It is always something that can be done in the future if need be. Take care, David

[Planting]

I didn’t have one and was diagnosed in 2017 ET jak2+. Seems some do some don’t depending on the specialist

[Orangeboykitty]

I was diagnosed through a blood test that showed I was JAK2+. At the beginning there was no need for a BMB. After a number of years my symptoms changed so my hematologist ordered one. Five years later I'm on a new medication (Besremi) so the hematologist ordered another one. Guess he wants to see what's happening on the new med. By the way, this time I had sedation for the test because the first time with just a local it was pretty awful. This time it was a breeze.

[HazeBlue]

Yes . If you tested positive Jak2. Then no need for bmb is what I was told. Are you on treatment?

[Katy121]

I had ET for 10 years before I had a BMB and it was not a particularly fun procedure. It showed I had some mild fibrosis, so started Pegasys treatment after the results were known. I am due to have a second BMB in June to see if the treatment has worked and reduced down the fibrosis. I would only have a BMB if it were needed for further diagnosis, otherwise I would not want to undergo the test. The test was uncomfortable and it was painful for the next 2-3 days.Maybe discuss is further with your consultant if you really feel it would be of benefit for you.

[Threelions]

Some mixed answers so far as there are clearly different reasons for consultants to ask for one. As far as I know it’s normal practice to have one a few years after diagnosis if one wasn’t done at the time of diagnosis. Had mine a few months ago approx 2 or so years after ET diagnosis (bloods ok at time) I was told it was to get a baseline reading of any fibrosis. My results were ok & in line with ET.& no further/different action required. It’s a fairly quick procedure and, whilst feeling a tad unusual, is not painful due to the effective local anaesthetic.Glad I had done as result was re assuring. Also if result had been different I’m guessing that meds might have been changed to my advantage so either way it’s a worthwhile procedure imo. I guess your consultant may ask for one in due course.

Good luck & all the best , Mark

[10061950]

Hi Bmj63I’ve also got et mpl and at the moment I am on hu and clopidogrel

and it’s never been mentioned to me to have a bone scan.

Iv seen a lovely Professor at Christie’s and he explained everything to me which was very useful I was really worried 😟 but he put my mind at rest.

I suggest you get a 2nd opinion and see a mpn specialist that’s what I did

ask your dr to send you for a 2nd opinion.

Hope this helps you. Good luck.

[Hopetohelp]

I’ve never had one

[Solyesh]

I had one to confirm diagnosis (ET Jak2+). In my case it was really to make sure we are dealing with ET and not pre-MF or masked MF - so while very uncomfortable I was glad to get the additional information. Also my MPN specialist advises getting them done for both diagnostic reasons and to have a baseline for future - basically we can compare any changes (if necessary). Now that I have changed to Peg will also be interesting in future to see what effect the INF has had on the bone marrow.

[BlastedKnees in reply to Solyesh]

I had a similar experience. Hematologist did initial test that found I was JAK2+ with high platelets, and said cut and dry case of ET. After reading here and other places, I saw a local MPN specialist and she strongly recommended a BMB to validate the diagnosis, and to get a baseline of any fibrosis to monitor changes. The BMB was not a bad experience for me, no pain just some needle pushing.

However, the results of the BMB changed the diagnosis from ET to pre-MF. If you read about it, there is some debate whether pre-MF is in fact a valid carveout of ET. My MPN specialist said that for me, it's essentially the same. I take aspirin daily, adhere to a healthy diet, get plenty of exercise, and finally, watch closely for changes in constitutional symptoms. I have very few symptoms currently, only some joint pain and occasional fatigue but nothing I can't push through. But she wants me to alert her of any constitutional symptoms - night sweats, bone pain, bruising / bleeding, etc. at which time she would further test for progression and recommend any changes to treatment.

For those who may be curious, I'm pasting the pathology results from the BMB that diagnosed pre-MF below. There's a lot more information in the overall BMB but this is the summary, and might be interesting to you if you're a data person (I am) and have wondered about what additional info a BMB may give you.

----

FINAL DIAGNOSIS

A. and B. (LPIC) Bone marrow biopsy and touch prep, aspirate smears, and peripheral blood smear: Normocellular marrow with mildly increased megakaryocytes with atypia, known JAK2 V617F mutation and thrombocytosis in peripheral blood, consistent with a myeloproliferative neoplasm. No increase in blasts or significant fibrosis (MF1 reticulin score).

INTERPRETATION:

The patient's history of thrombocytosis, possible essential thrombocythemia, is noted. The bone marrow core biopsy shows normocellular marrow and mildly increased megakaryocytes with occasional large forms with cloud-like nuclei. No large clusters of megakaryocytes are seen. Reticulin stain does not show increased fibrosis (MF-1).

Overall morphology favors primary myelofibrosis in pre-fibrotic phase. The bone marrow aspirate smears show progressive trilineage maturation. Increased megakaryocytes with similar morphology to the ones seen in the core biopsy. The bone marrow core biopsy touch imprint shows similar findings to the aspirate smears.

BONE MARROW CELL DIFFERENTIAL: (percent of200 cell count on bone marrow

aspirate smears)

0 Blasts

4 Promyelocytes

7 Myelocytes/Metamyelocytes

37 Bands/segs

2 Eosinophils

33 Normoblasts

14 Lymphocytes

3 Monocytes

0 Plasma cells

CELLULARITY: 50%

M:E ratio: 1.6:1

[EPguy in reply to BlastedKnees]

Interesting, your result looks relatively mild, for example "Normocellular marrow" but its Dx is pre-fibrotic.

Your lab states that MF-1 level is not increased fibrosis. But the scale starts at 0, so does that mean 1 is equivalent to 0 for that purpose?

You got some CBC looking results (ie Lymph etc) I assume that was from the blood sampling they included. My BMB list does not have these.

--

For reference my summary looks like below. The fibrosis level is mathematically written as not exceeding 1/3, but I think it means "out of 3".

The last line "correlation" is where I got my primary PV Dx along with the ET features. This difference shows that the BMB may not be the last word in our Dx.

CLINICAL INFORMATION

New diagnosis of MPN with JAK2 V617F mutation. Initially presented with abnormal blood count (thrombocytosis and increased absolute neutrophil count).

FINAL DIAGNOSIS

BONE MARROW, ILIAC CREST (PERIPHERAL SMEAR, ASPIRATE SMEAR, TOUCH PREP,

CORE BIOPSY SECTION, CLOT SECTION):

- Hypercellular bone marrow with maturation of all cell lines. Megakaryocytes are increased with clustering and hyperlobated forms (see comment)

- No increased blasts confirmed by flow cytometry (see concurrent flow cytometry case)

- Focal mild reticulin fibrosis (MF 0-1/3) with reticulin special stain

- Iron stores are absent per iron stain

- Peripheral smear shows marked thrombocytosis

COMMENT:

The findings are consistent with a myeloproliferative neoplasm, and suggest essential

thrombocythemia (ET). We note the presence of a JAK2 mutation in a previous study. Correlation with clinical exam findings and ancillary laboratory results is required.

[BlastedKnees in reply to EPguy]

The CBC looking results (blasts, lympho, etc.) were from the BMB lab. They did a separate CBC at the same time. My CBC results are generally good with the only real issue being slightly high platelets, ranging from about 470 - 530, which in MPN-land is pretty mild, only a hair above normal.

To your point, there's no fibrosis yet which is valid for MF-0 and MF-1. I had to look this up to figure out the fibrosis scale. Difference is when scarring occurs (MF-2 and MF-3), represented by collagen bundles:

Pre-fibrotic primary myelofibrosis (MF-0): scattered linear reticulin fibres with no intersections.

Early-stage primary myelofibrosis (MF-1): loose network of reticulin fibres with many intersections, especially in perivascular areas.

Fibrotic stage primary myelofibrosis (MF-2): diffuse and dense increase in reticulin fibres, with extensive intersections and occasionally with focal bundles of collagen.

Fibrotic stage primary myelofibrosis (MF-3): diffuse and dense increase in reticulin fibres, with extensive intersections and coarse bundles of collagen.

I had a good chat with my MPN doc about what in the data is pushing towards the pre-MF diagnosis. She said it's a combination of things - the numbers and types of cells such as the increased megakaryocytes with cloud-like nuclei, and MF-1 fibrotic level. The rationale wasn't very satisfying to me, and my reading about pre-MF didn't give me much more clarity.

Studies are inconclusive on whether pre-MF is actually a valid diagnosis (vs ET) and there are arguments ongoing; The more I read the more fuzzy it seems. Everything is a continuum and is evolving quickly, so I just take my aspirin and see the doc for tests occasionally - effectively treating pre-MF like ET.

[EPguy in reply to BlastedKnees]

Thanks for the details. From these definitions, the most obvious divider seems to be presence of collagen fibers, which I do know are a more advanced condition. In that case, Fibrosis and Scarring are both specific to collagen type fibers.

But an additional factor from your MPN Dr is the cloud like nuclei. So maybe it's collagen + cloud nuclei to have Pre-MF? That's at least a sort of definition.

I agree on the vague Pre-MF, nothing I've read gives a solid definition; maybe the one here is something. But our conditions are all about degrees and uncertainty.

[EPguy in reply to Solyesh]

I agree on the baseline for future. We may have access soon enough to therapies or combos where fibrosis reduction is a specific endpoint. We don't yet, but if/when that happens, we'll be glad to have that early reading.

[Bears23]

I had bmb, and many years later I required a second one. The MPN specialist was very pleased I had the first one and used that to verify progression. I personally didn't have any issues with the bmb (both tbmb ook about 3 minutes and I felt no pain (just a bit of pressure), and it was done right in the office. But, we are all different andd some find the bmb uncomfortable. Best of luck to you!

[Beup]

Hi!I was recently dx with ET by a local hematologist. I then saw a MPN specialist a couple weeks ago and she wanted a BMB that day to confirm dx for future tx. I had been thinking up to that point to get one just for more information and as a baseline. I asked for their most seasoned person to do the procedure, and she had 20 years experience doing a few a day. It was fine and pain was brief at a level 2 scale of 10. I think the procedure requires finesse and skill and patience. I like the idea of my experienced MPN team reading and interpreting the results.

There is a part of me though that wonders if ignorance is bliss.

[ciye]

I was diagnosed the same way you were.

[Janis12]

Hi, I was given the choice of having one as I was negative to the regular mutations so it was the case of being more conclusive. Diagnosed with ET 2 years ago and if I have to have another BMB in years to come I would be okay about it and that's coming from someone who is a bit of a chicken when it comes to medical stuff.

[Vtr1000]

Hi Bmj63, when I was diagnosed all the blood tests validated my MPN. I was offered a BMB for a baseline for the future. It was left entirely my decision.

[Meatloaf9]

Hi, I had a BMB in 2018 to confirm the diagnosis and to have for future comparison to determine progression. Both my regular hematologist and mpn specialist recommended the BMB for those 2 reasons. The BMB is supposed to be diagnostic. However, I was told I had ET based on labs and the BMB but when my Hct jumped to 55 last year they told me that I most likely was always PV, now my Dx. I felt no pain whatsoever during the BMB with local anesthetic. The hematologist who did the BMB had 40 years experience, I think that makes a great difference. Best to you with your decision.

[Rockstone]

My haemotologist suggested having a BNB, I said I didnt want it and she said ok no problem!

[krotsida]

Hi there I too have ET Diagnosed in 2017 and have never had to have a bone marrow biopsy. All the best.