triple negative ET

[Carthe]

… I’m a bit confused with the triple negative - does this mean they can’t find which gene it is but the bloods show it’s an ET or does it mean they can’t see from the bloods if it is an ET or the gene…But because you have high platelets they treat it like one? 

[Cat1001954]

I have same as you and I believe it’s rare to have but I also don’t really know what it means

[Cat1001954 in reply to Cat1001954]

and never enough time in consultant. Appointment to ask

[hunter5582]

The short version is that triple-negative mean the person meets diagnostic criteria for the MPN but does not test positive for one of the known driver mutations. Here is a bit of information on this topic.

ncbi.nlm.nih.gov/pmc/articl...

researchgate.net/publicatio...

youtube.com/watch?v=Oc8ujrS...(Not working as expected?)

[Carthe in reply to hunter5582]

urgh that’s depressing - and I don’t understand all of it 😢

[Carthe]

I just so scared it it comes back negative then what is it? Have I been harbouring something that they just having diagnosed yet? I’ve no idea how to get through this it’s all too much

[hunter5582 in reply to Carthe]

We each find our own answer about how to deal with it. It helps to know what "it" is. Fear of the unknown if often worse than fear of the known. In addition to developing a coping skills list, I have found several things very helpful.

1. Feeling powerless causes fear. Knowledge is power. Feeling empowered effectively reduces fear. Once you know where to focus your learning efforts, learn what you need to know to manage whatever "it" is.

2. Managing fear is also about finding a way of thinking about it that works for you. I found an excellent resource in a book by Adm. William McRaven, Make Your Bed.

3. When facing fear, I like to use this quote to set the right mindset. "I must not fear. Fear is the mind-killer. Fear is the little-death that brings total obliteration. I will face my fear. I will permit it to pass over me and through me. And when it has gone past, I will turn the inner eye to see its path. Where the fear has gone, there will be nothing. Only I will remain." Frank Herbert, Dune.

Know that you have this. Have confidence you will understand what is going on and will make choices to manage it effectively.

All the best.

[Jelbea in reply to hunter5582]

Thank you for your words on "fear". I have copied this and feel it will help me a lot. I have suffered from generalised anxiety for a long, long time and try hard to overcome all the "fears" which spring to mind. Not an easy task.

[CalL1981 in reply to Carthe]

I am triple negative (negative for Jak2, MPL, CALR). They also ran Next Generation Sequencing which did not show any other mutations. I have had two BMBs which both confirm ET diagnosis. Had they found a known mutation it is likely they may not have done the BMB. The reason for the second BMB was I moved countries and the new hematologist questioned the MPN diagnosis given the lack of mutation. Following the second BMB they came to the same conclusion as the first hematologist. My understanding is that it is possible there is a mutation that hasn't been linked to MPNs yet and is not yet known. Have you had a BMB to confirm diagnosis? I understand it is all very overwhelming but you will feel calmer with time.

[lizzziep in reply to CalL1981]

I’ve just had bloods sent for Next Generational Sequencing, wasn’t sure what they did - I know I’m JAK2 negative so now you’ve said that I realise what they’re looking for. (Although I was hoping for a part on Star Trek!)

[Wyebird in reply to Carthe]

they find out more and more about MPNs yearly even daily.

I have Calr ET.

I have the mutated Calr gene.

It wasn’t discovered until until about 10 -15 yrs before my diagnosis.

I have very high ferritin levels but they have found no reason for it. I was scared about that but just have to accept it.

Look on the positive they are treating and monitoring you.

Try and relax and enjoy life. Any tiny question or worry post it here someone is always on hand to help💝

[Wyebird in reply to Wyebird]

I stand corrected 2 yrs before my diagnosis the mutation was discovered.

[Carthe]

I haven’t had a bmb the haematologist said he wouldn’t do one as it’s too invasive. He would prefer to watch and wait. I’m still ok the fence - I have no symptoms and my platelets peaked at 468 a couple of weeks ago and are now back at 435 although they were 438 in September 2018…. So I’m wondering if stress can impact them as I have had chronic stress since then and am probably depressed too… if I’m honest…

[MaggieSylvie in reply to Carthe]

Stress can influence inflammation and inflammation can affect the platelets. But in any case, when we have a blood test, it only shows the figure they are on that day. The next day it is likely to be different. As I understand it, if your platelets are less than 500, they are in range. A difference of 3 is just a little bleep and that would be what they were on that particular day. Don't let these figures depress you. You have no symptoms and you don't have to go through a BMB - Yay! So just get on with your life and get rid of your depression any way that is appropriate for you.😊

[SuET2017]

I am ET triple neg, which means I just don’t have one of the drivers that has been discovered/identified at this stage. Being triple negative makes no difference to treatment options. i am treated the same as anyone else with ET. I am on medication to lower platelets (pegalated interferon) as my platelets go to over 1000 when I’m not. On treatment they sit around 450 which is a more than acceptable number. I hope that helps

[lizzziep in reply to SuET2017]

Do you have any side effects with the peg interferon? I think that’s what I’ll be changed to next month. Been on Anagrelide for a few years.

[SuET2017 in reply to lizzziep]

some (little bit of hair loss, flushed dry skin) but nothing significant. For me it’s been the best treatment. I had awful side effects on hydroxy and anagrelide.

[lizzziep in reply to SuET2017]

Thank you. The Anagrelide side effects haven't been too bad, hydroxy caused ulcers on my feet!

[SuET2017 in reply to lizzziep]

ouch! It gave me heart palpitations- like drinking a quadruple espresso! And I was permanently nauseous on hydroxy.

[MaggieSylvie]

I am triple negative. It means I don't appear to have any mutated genes, and the triple refers to the well-known three JAK2 etc. It doesn't mean you don't have a mutated gene that has caused ET but it hasn't been detected yet. It's probably a good thing to be triple negative because if you have one or more of the famous three, they can develop into something else. That's my spin on it, anyway.

[IDRE]

Has your doctor ruled out reactive thrombocytosis instead of essential thrombocythemia? Triple negative ET is more rare from what I have read (I read a lot although I recognize I have only a limited knowledge.), but still possible. My doctor went the route of blood work and bmb to confirm ET (I have the JAK2 mutation) before exploring other possibilities that could have been causing high platelets.

[Carthe in reply to IDRE]

Thank you for your reply? I have had all the tests done autoimmune etc and they are all negative…. The only thing I can think of is my chronic stress levels have been through the roof over the past 5 years… are there any other tests I am missing? X

[IDRE in reply to Carthe]

Stress can definitely affect your general overall health and I have read that it can also affect platelet counts. Infections, low iron and other conditions can affect platelet count too. A complete blood panel can provide clues to the doctor and should be part of the overall diagnosis. The only thing I can recommend is to find another doctor if you don't feel comfortable with the lack of diagnosis. It is important to have a medical team that you can talk with and that you feel are doing everything to find out the whys. And try not to let a diagnosis of high platelets add more stress to your already stressful life. I know...easier said than done, but it is important to learn how to manage stress for the benefit your overall health now and in the future. And there are medications that aid in lowering the platelet counts. I wish you the best!

[Carthe in reply to IDRE]

I’ve had bloods done and there is nothing out of range other than platelets hb was slightly higher at 156 and hct at 47 but they said that was in range - he wasn’t concerned about pv said he was looking at et …. But platelets at present are 436 …

[Carthe]

Thank you for your reply? I have had all the tests done autoimmune etc and they are all negative…. The only thing I can think of is my chronic stress levels have been through the roof over the past 5 years… are there any other tests I am missing? X

[EnergizerBunnyz]

Same situation here, with CSR in high 80s. So there is inflammation, but all organs testing came out normal! What does this mean to have ET but none of the 3 mutations (CALR, JAK2, MPL).