I've posted a few times and still in no man's land (hopefully it will get better on my second haem appointment on 22nd).
So far: ET possible - no secondary causes for thrombocytosis (everything normal which is great) ;platelets hovering around 500; previous pulmonary embolism at age 18 - 20 years ago; jak 2 negative ;awaiting CALR and MPL results; spleen normal;BMB stated as morphologically normal - shows no blasts (<1%), normal maturation of all myeloid cells and no atypical megakaryocytes but notes say 70% cellular (seems slightly high given age is 40?) and grade 1 fibrosis normal. I'm wondering where on earth this will go with diagnosis and wondered if anyone else with normal BMB had an ET diagnosis? Also, I thought grade 1 fibrosis is minor changes not normal per se but I guess it depends what grade classification they use for fibrotic changes (some start at 0 and some start at 1). I've left messages for haem to have some confirmation before appointment but don't seem keen to get back to me. The waiting is horrible.
Sorry for rant - any comments welcome (I also realise that all considered I'm very lucky)
Eve
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RedSunrise
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First good thing is that your appointment is coming up quickly so you won’t have to wait long for answers. I’d recommend writing down all your questions; when we’re sitting in the doc’s office, sometimes we forget to cover something we wanted to know. I’d write the doc’s answers down too! Second thing - I know my haemo was quite certain I had ET before I had my BMB which showed things “consistent with” ET. In your case, the answer may be different as your results are showing a lot of “normal” other than the elevated platelets. And they’re not super-high so try not to worry (easier said than done I know) and you’ll have your answers in just a couple of weeks.
I get days when I'm calm and then as soon as I get more results it opens up the whole stress again. This started 6 months ago. I realise I'm lucky but I'm concerned this could be the very start of things; also concerned given previous embolism.
Thanks for tips - I had a huge list of questions for haem on first appointment and intend to have a longer list this time around!
Thanks again for your reply. I'll try and calm myself down.
I had a fairly normal BMB in the beginning...mine was hypocellular and the only thing that was pointing in the direction of ET was I had a few megakaryocyte clusters. Megakaryocytes are the cells responsible for breaking apart and becoming platelets and they tend to cluster in ET. Other than that it was normal. They wouldn't make a diagnosis of ET from my result. I didn't get a diagnosis until many years later when I was tested for the CALR mutation. And my platelets have never been over 533,000 in 15 years.
How annoying, I did actually respond earlier but the message didn't send for whatever reason.
Thank you for responding. How did you find the wait in between? May I ask what prompted you to have your CALR test many years later? Were you on watch and wait or 'discharged' from haematology in between?
I was frustrated at the lack of diagnosis. My first hematologist wasn't a very nice guy and I just walked away from the whole thing. The CALR mutation hadn't been discovered yet. I kept an eye on my platelets over the years and noticed they weren't really getting higher so I kind of let things go.
Over a decade later I became iron deficient and that raised my platelets even higher and that's when my primary care doctor noticed and sent me to a different hematologist. That's when I tested positive for CALR. I did have more symptoms at the point such as ocular migraines, blurry vision, and cyanotic fingers and lips. We treated my iron deficiency and my platelets returned to their normal high level. Currently I haven't seen a doctor nor checked my bloodwork in over a year because I'm over the whole thing. I don't dwell on it too much.
I hope you find an answer soon as I know how frustrating not having a diagnosis is.
Hi Tina. 🤗 I have to admit I haven't looked into it yet. I don't have good health insurance right now and I can't bring myself to spend a few hundred on a doctor's visit when I'm feeling ok right now. How have you been doing?
Been having searing pains in the back of the head when waking. Gp gave me meds for migraine but not got any usual symptoms of them, got a review with him this evening. Still getting them,didn't mention them to haemo when i saw him cos his ignorant behaviour on entering his office p***ed me off, ie, taking a personal call. tina.x🤗
See, that's the crap I can't stand anymore. I have more bad experiences with doctors than good experiences. I've gotten to the point that I just avoid doctors because it makes my depression worse. One of the last times I saw my hematologist he entered the room silently and proceeded to turn the computer on. He stood there for 5 silent minutes with his back turned toward me with not even a hello. Once he actually did speak to me he was quick to blow off my report of cyanotic fingers and I was so done at that moment and I said no more. I have so many stories of bad doctors. I mean I know my disease is not as serious as the person in the next room with pancreatic cancer but I still deserve respect and I'm still paying for the appointment.
I'm sorry about your head pains. I hope you can figure out what it is. 🤗
You are spot on about that experience with haemo, couldn't even show any courtesy and giving a good morning.Costs nothing! Will see what gp says tonight. Will keep you updated.tina.x🤗
Sounds like a horrible experience with haems - I'm sorry to hear that. That's why I'm so 'on this' even though I'm clearly not as ill as others. I don't want to get sidelined and fobbed off. My haem was pleasant enough but I have doubts over some of the things they said (sounds very arrogant I know due to all the training they have had - and who am I, lol!) I will however persist with questions and be persistent in my polite challenging. If I'm not happy I will ask for a second opinion. Ultimately, they are obviously very well trained and extremely intelligent but it doesnt mean they are all experts in MPNs and they aren't personally going through this waiting experience. It is easy for them to become desensitised (and to a degree it is necessary for them to do this to perform in their role for the better of all their patients). Whilst I will be respectful towards them because of their profession, I will not hold them on a pedestal - they equally have their flaws like the rest of us humans.
Gosh, sorry! Rant over (how cathartic though!) . I'm going to try and forget about all this debacle until my next haem appointment on 22nd for the sake of my sanity!
I hope that you find some better support. It's truly terrible the rudeness you experienced and I don't know how I would have dealt with that.
Hi Eve. You are doing the right thing. With your history of PE you have to stay on top of things. You are correct about doctors having flaws just like the rest of us. I worked in the veterinary field for 19 years so I have worked side by side with many doctors and I can say there are good doctors, bad doctors, and mediocre doctors. I definitely worked with a few doctors I knew I would never let touch one of my animals. And let me tell you whenever a client brought their pet in to see one of those bad doctors I felt so bad for them and just wanted to take them aside and say, "Psst, you may want to change doctors." But I never could say that. I think in the future when I find a new doctor I'm going to ask the receptionist which doctor she would use! Please let us know what you hear on the 22nd!
Thanks for the good advice. Whilst what you say is deeply saddening, I agree whole heartedly and I think it equally applies to every profession. I've not worked in the medical profession but I've been a financial advisor and same rules apply.
No doubt I'll have some more rants on the 22nd! Hopefully not!
Exactly my situation. Years on and still a presumed diagnosis. We can’t find a secondary cause, I am triple negative, platelets currently 826 (high since at least 2006). My biopsy was mostly unremarkable with mildly increased fibrosis (didn’t give a grade). I am very symptomatic and very weary of the whole thing! It’s hard not having an official diagnosis because I always have to wonder if we could be missing a secondary cause that is behind my symptoms.
Thank you so much for providing all that information. The unknown is definitely stressful - whilst I certainly don't want to be ill, I don't want to be left wondering - exactly like you say. May I ask then you say a presumed diagnosis; so they've said presumed ET? does that mean you could claim on critical illness cover for example or how do you disclose for travel insurance?
Interestingly, I've literally just had my old NHS file notes sent to me from when I was admitted to hospital at 18 for a pulmonary embolism to look at my platelets from this time. They show them elevated at 483 then which was 20 years ago and, of course, I had a clot! On my first visit to the haematologist I stated that presumably on diagnosis I would immediately be placed at high risk given the previous embolism. She quite emphatically said no as she felt it was an unrelated incident. She did not have my file going back to 1997. However, now that I have these platelet results going back that far, how can it be anything but related to my current situation?!It's really worrying regarding clot risk and potential progression.
How do you manage to cope if this has been going on since 2006 for you? I feel like I'm starting to become a rather neurotic person which is something I've never considered myself to be before!
I have only known since 2016. After pulling all of my records we were able to see that all of my CBCs since 2006 were elevated. In 2001 the level was 398 so somewhere between there is likely when it started.
It seems strange for them to brush off the embolism like that. Can you get a second opinion?
By “presumed” diagnosis I mean that there is no other explanation so my hematologist presumes that I have ET but I have no diagnosis. Medically, he codes everything with the diagnosis code of ET.
I’m in the US so I’m not sure about coverage.
I understand feeling neurotic. I get very frustrated about it. For the first year I was especially anxious about it. Now I’m at my wits end. I have a second opinion visit scheduled for April 1. I’m hoping to get platelet lowering medication to see if it will help my symptoms but my current hematologist is not willing to try.
We gave previously been in contact on this trail and I've noticed you replied to someone recently and said you've had a diagnosis. I think we were in a similar situation I. E triple negative and an unremarkable BMB. May I ask what your diagnosis was and what made them arrive at that diagnosis?
Hi Eve! I switched hematologists and they repeated the bone marrow biopsy. The biopsy showed an abundance of megakaryocytes and another marker. The biopsy itself said something like “highly suspicious for essential thrombocythemia.” Combined with all the secondary causes we’ve ruled out it was good enough for the hematologist to make a diagnosis even without a mutation.
Have you made any progress trying to sort yours out? There must be another mutation or cause for many of us that I hope will be discovered soon.
I'm glad that it sounds like someone finally listened to you as it sounded like you had been badly overlooked. I take it the diagnosis has led to you having some different medication to reduce symptom burden? How are you feeling?
My official diagnosis was no diagnosis with 6monthly monitoring. I'm taking low dose aspirin to reduce risk of clots. To be honest I'm happy at the moment to have some peace and stop reading so much (I just get the odd notification from this site) . I'm concentrating on getting fitter. However I was thinking of asking for a second opinion on my next bloods as I wasn't happy with the haemotologist at all. I just don't understand how I can have high platelets, have had a clot before (with high platelets then) and have some fibrotic changes with slightly high bone marrow cellularity and all the haemotologist kept saying is my BMB was normal. I have requested all my file and will push for second opinion in August.
Did you have to have a second biopsy or did they use your last sample? They told me that some of my sample would be stored. Do you mind me asking what the other markers were that showed up this time around?
I would keep pushing, but I know it gets exhausting. It’s hard to trust your treatment plan when you don’t know the cause for certain.
I had to repeat my biopsy. My first one was a very tiny sample size (less than half a cm in length) so they wanted a new one. They started me on Hydroxyurea. My energy levels have definitely improved but most of my symptoms are still here. I’m hoping I’ll continue to see reduced symptoms. I have only been on it for about 5 weeks.
My biopsy says under diagnosis: “Marked thrombocytosis”
“Hypercellular (80%) trilineage bone marrow with atypical magakaryocyte proliferation; features highly suspicious for myeloproliferative neoplasm. See comment”
In the comments it says, in part “Megakaryocytes are increased in number with focal loose clustering and enlarged mature morphology. The overall appearance in the context of thrombocytosis suggests a myeloproliferative neoplasm, in particular essential thrombocythemia”
So I guess that combined with years of specialists ruling out secondary causes and at least 13 years of high counts is good enough to be diagnosed.
Thank you so much for sharing all that information. I guess it's a double edged sword - of course no one wants to have a diagnosis of this sort but with the diagnosis comes relief of not being left in no man's land. Also, you can get the right treatment. That's a real positive about energy levels, hopefully some of your other symptoms will improve the longer your on the medication. Fingers crossed for you.
I really admire your perseverance to push for a diagnosis and its paid off for you to start to feel better. And at least your BMB seems relatively good (like mine). May that long continue.
Yes, I will push. Reading your report winds me up about my BMB and I'm not sure who wrote the snipping of the BMB report I saw I. E was it a full report or was that my haematologist summary of my report. My symptoms don't seem to be as bad as yours. I did have a lot of fatigue and started to feel a bit depressed but I've started pushing myself the last few months to exercise and try to put this to one side for a little while, at least until my next bloods. However I'm still not as sprightly as I used to be in the mornings. Tingling in my hands and the ringing I used to occasionally get in my ears seems to have stopped since taking aspirin though.
May I ask how old you are? There is something called age adjusted bone marrow cellularity and mine is just above that.
It' such a shame that you had to go through your records that far back - it's amazed me that 'the system' doesn't just collate all this information (same for me in UK) and, more worryingly, that this wasn't highlighted earlier for you.
Once I have my second haem. appointment, if I'm not happy I will most certainly be asking for a second opinion.
I'm so sorry to hear how you are feeling - I can completely empathise and you've been going through this debacle longer than me. I really hope your visit on 1 April goes well and that they can give some medication to relieve symptoms. I take it you are just on aspirin at the moment - I can understand your desire to try something to actually lower the platelets given your symptom burden.
Let us know how you get on. I shall follow you on the site as I have an awful memory with names.
I was in a very similar situation and it was only the positive CalR test that gave the confirmation. At the time it was only recently discovered so the haem was keen to test for it.
With regards to medication, if it was me I would stay off it for as long as it’s safe to do so. I have had very few symptoms so have been on a watch and wait course of treatment. It’s only now that my platelets are at 1600 that we’re discussing medication.
For travel insurance I have always declared ET and it has had no effect on the premium even when I went skiing in Canada and travelled to Oz (I live in the UK) despite having to have fragmin injections for the flight.
I think that one of the big things I had to come to terms with is the uncertainty and the amount of unknowns - it gets easier with time but it doesn’t make it better.
I hope you get some answers soon and that it helps you make sense of this all.
My only unsolicited advice would be to find something that you enjoy and make it part of your life - for me it was yoga, helps to take my mind off of it all for a short period of time. That and drink lots of water - dehydration makes me feel awful!
I meant to respond to your reply sooner. Thank you for the advice.
I'm starting to realise that I need to focus on another distraction (you're right) I need to get going with my exercise instead of sitting down (eating) and incessantly reading about this condition! I also don't drink enough water (bad habit!).
I hope meds go well for you. I agree with staying off them for as long as possible. I just want to make sure that with regards to risk stratification that everything is being done to ensure I'm put in the right risk group.
Thanks again (also good news about travel insurance!). Hope you have some more, lovely holidays planned.
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