First, thank you to all who have reached out to me this last week since I joined the forum. Your kindness has truly helped me come to terms with all this.
I have my first meeting with my MPN specialist coming up soon. He will be giving me the results of the genetic testing to see which driver mutation I have. At this point, all other causes of high platelets have been ruled out, and I do not meeting the blood count criteria for PV, so I have been diagnosed with ET by exclusion.
I am 30 years old, and my bloodwork indicates I have likely had ET for 10 years already with my platelets showing stable between 440 and 450 since 2011. Should I push for getting on pegylated interferon? My understanding is that I am "low risk" as I have never had symptoms and my platelets are very low, but I have heard that interferon can slow or even (rarely) reverse disease progression, which is my primary concern having this at such a young age. Progression scares the life out of me.
Any suggestions would be great. Thanks!
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I honestly wouldn't start with anything before properly diagnosed. If genetic tests come back negative (or positive for any driver mutation), I would ask for a BMB. If this really confirms ET you have to discuss your options with the specialist.
I've read so many stories about continouos high platelets and some people were just perfectly healthy, although there was no apparent reason for elevation. Maybe in your case this could be the same?
I’m still a newbie here, but my understanding is that the absence of driver mutations would be someone who was “triple negative” and diagnosed with ET out of exclusion of other causes. ET can still be diagnosed with or without a driver mutation. Having elevated platelets above 450 and eliminating all other causes (inflammatory disorders, underlying causes, etc.) is still grounds for ET, even if they can’t find a cause.
If your BMB comes back normal und you don't have any driver mutation, you don't have ET. If you don't have any driver mutation, but your BMB indicates ET, you have triple negative ET. Maybe it's just the way you are?
I don't want to rule anything out (I'm not a doctor - at least not for humans ), but I wouldn't say you definitely have ET as long as there is no proper diagnosis. I'd like to hear what's the outcome of your appoinment. I wish you all the best!
This is very good to know, thank you. My hematologist (not the specialist) told me people can still be diagnosed with ET even with a clean BMB since it is a “diagnosis of exclusion”. I will keep this in mind. Will let you know how it turns out!
Never heard about that as ET is classified as cancer which has to meet certain criterias. And there are more than just "elevated platelets".
But as mentioned above, I'm not a hematologist. I wouldn't worry to much though. I know it' easier said than done, I went through all this emotions recently and still do. In my case there was no happy end, but it doesn't mean there can't be one for you.
I guess what I’m really looking for here is an answer as to whether interferon would be better than “watch and wait” in my specific situation assuming the genetic testing reveals a driver mutation, which is what both the specialist and hematologist have told me is the far more likely outcome. Is starting interferon now at 30 to prevent progression the better option to “watch and wait”.
Genetic testing is important. However BMB will tell whether megakaryocytic hyperplasia in bone marrow as well as cellularity and fibrosis level. Normal population could have JAK2 mutation, MPN patients could be mutation negative due to low allele burden or the gene mutation is not discovered yet.
In the diagnosis of Primary Et the majority will have Bone Marrow cellularity and Megakaryocytes Hyperplasia present. If your Bmb came back normal but you still had persistently high platelets it would be termed secondary Et, than your Haematologist would be looking for other causes, more than likely inflammation issues. Tina.🤗
Bear in mind that the WHO cutoff is actually 450. Diagnosis of ET requires additional markers. mpnconnect.com/pdf/who-diag... .
You do not know yet whether you have ET. However, we all understand the worry. If you do have ET and are in the low-risk group by age and lack of symptoms, then standard protocol is aspirin-only and monitor. That is a good plan if you are young and have low-risk ET. That is how I spent the majority of the last 30 years, even when the ET "progressed" into PV.
JayneWayne is correct in stating that a BMB can be helpful in establishing the diagnosis, but not all docs do them all the time. There is another test you can do the help establish your risk profile if you are positive for one of the driver mutations. This is a MPN Myeloid Panel such as integratedoncology.com/test... .
Bear in mind that most people with ET do not progress. You are more likely to die with ET than from it. Not to say there can't be issues (e.g. systemic inflammation) - but those can be managed.
Glad to hear you have the appointment with the MPN Specialist soon. He/she will go over all of this with you.
« Interferon should be considered as upfront treatment in all newly diagnosed patients with ET, PV and hyperproliferative myelofibrosis, the aim being to treat a cancer from the very beginning to prohibit cancer progression from early cancer stage (ET/PV ) to the advanced cancer stage – myelofibrosis – when immature cells -including stem cells ( CD34+ cells ) egress from the burning hell ( the bone marrow niches – “the chicks flying prematurely from their the nests” – ) to the circulation to seed ( metastasis ) in the spleen, the liver and elsewhere .
The world is divided in two – the one having access to IFN and the one not having access to IFN.
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), but I wouldn't say you definitely have ET as long as there is no proper diagnosis. I'd like to hear what's the outcome of your appoinment. I wish you all the best! 
Pegylated Interferon...starting soon
Starting Interferon
myelofibrosis and interferon
Interferon journey started with ET JAK+ at platelets counts 550
feeling quite fed up, is it possible that interferon doesn’t work for me? 
