Possible Essential Thrombocytheania?

[bluebird567]

Hi friends,

I have just joined the fantastic organisation for help with MPN's. Sorry this is a long post but I would appreciate any help or advice you can give.

In the last 6 - 9 months I have been feeling exhausted after doing some work on my allotment (not a lot) or gardening, or housework etc. I thought it might be my remaining parathyroids not working like they should (I had two removed December 2020 after 18 months of feeling exhausted).

I ask for annual bloods due to my previous nephrectomy and Hyper- Parathyroids disease. I had noticed my platelets numbers rising over the last few years but when I mentioned this fact to the GP then, I was told they were still within what was acceptable, even tho' over 450,000.

However, the last results showed plates to be 600+ and a new GP wrote to heamatology for advice and an appointment was made with them.

I saw a registrar who had my results in front of her and asked a few questions. She examined my tummy, groin, armpit and neck areas and ordered more bloods which I believe will be more thorough. These will take 3 weeks to come backand she said I would have an appointment in 4 weeks. My appointment is actually in 7 weeks. She also gave me a leaflet on HYDROXYCARBAMIDE also known as Hydra (a capsule form of chemotherapy) and said she suspected a blood cancer.

I was somewhat numb and didn't know what to ask. I went home and started looking at Dr. Google, not the best advice, and came up with ET.

I have now noticed more bruising on my legs, I didn't think they were anything to do with platelets and didn't mention it to the doctor, but now I am unsure.

If anyone has information on questions I can ask at my next appointment please let me know.

Thank you for giving the time to read thro' my post.

Bluebird567

[hunter5582]

Hello and welcome to the forum. Glad you found your way here. this is a great place to find support and information from a MPNer perspective.

What you are describing is consistent with a diagnosis of Essential Thrombocythemia; however, that is not definitive. Thrombocytosis can be either primary (Essential Thrombocythemia) or secondary (Reactive Thrombocytosis). Your case is significantly complex and to requires ruling out secondary causes. One of the more advanced blood tests you likely received was a MPN Myeloid panel that will check for the three driver mutations associated with ET (JAK2, CALR, MPL). Most people with ET will have one of these three mutations in hematopoietic stem cells.

ET comes with an increased risk of thrombosis (blood clots) and hemorrhage (bleeding/bruising). The reasons for the seemingly contradictory twin risk has to do with how the platelets behave. In addition, ET is caused by deregulation of the JAK-STAT pathway, which also causes the overproduction if inflammatory cytokines. This is thought to be the cause of many of the secondary or constitutional symptoms people with MPNs experience. Fatigue is the most common of all MPN symptoms.

It is shocking to hear that you may have a "blood cancer." It is important to put this in perspective. ET is a chronic disease, not an acute disease. Properly managed, most people with ET can expect to live a normal lifespan. Many of us look at it as cancer with a little "c" rather than cancer with a big "C".

I was diagnosed with ET over 30 years ago. It progressed to PV about 11 years ago. Now age 69, I have been living a good life and continue to do so. There have been some issues, MPN and non-MPN related, but they have all been managed. Treatment options for MPNs have improved considerably in recent years. I feel better now than I did 10 years ago.

There are several options to treat ET when cytoreduction is needed. the two first-line treatment options are hydroxycarbamide (AKA hydroxyurea - HU) and Pegasys. Before making a decision, it is important to consider all of your options, weighing the benefits and risks of each choice. Here is a bit of information about your choices.

mpnjournal.org/how-i-treat-...

nccn.org/patients/guideline...

It is important to consult with a MPN specialist rather than a regular hematologist. MPNs are rare disorders and most doctor, including hematologists, have little experience with them. Here is a list of MPN expert doctors. mpnforum.com/tsr-the-list/

Consulting DR. Google about ET can be unduly alarming. It helps to stick with reliable sources. MPN Voice is an excellent starting point. mpnvoice.org.uk/

There is some excellent training online. Here is one of the better ones. Suggest starting with MPN Molecular Biology. mpninfo.org/conferences/202...

Wishing you success as you move forward.

[bluebird567 in reply to hunter5582]

Hi hunter5582 and thank you for your kind and most informative information. I will check out all the links you have given and digest them one by one. I wanted to reach out to people like you who have so much more knowledge on the subject and thank you again for sharing this knowledge. Whatever my prognosis is, I know I'm in safe hands with MPN Voice and the wonderful people like yourself that contribute to it. I'm glad to hear you are so much better than you were 10 years ago. It all sounds so positive. Thank you again.

[TillyUtt66]

Hello please don't worry I have ET and hydroxycarbamide Clopidogrel and some other medication. I thought I'd got menopause at first then when my legs were hurting as in all down the bone so I could cry with the pain. But I was put on Clopidogrel first which helped bit then after a couple of months my bloods were going up like you. Then I was put on hydroxycarbamide then it was months of seeing what worked tablet amount now it's settled at two a day. But it's the Clopidogrel that causes the bruising so after a while you think oh that's going to bruise after you knock your arm , leg or whatever. Sometimes even having your bloods done leaves finger prints. Also tiredness is part of it and for me it ebes and flows.Please takecare

[bluebird567 in reply to TillyUtt66]

Thanks for your reply. MPN is a minefield and it seems everyone has different responses to the medications. I'm so glad that I have guys like you for excellent support and knowledge. I hope you continue to manage your drugs and keep well.

[lizzziep]

Don’t believe everything Dr Google says, most information is well out of date. It does sound like ET but tests should give a definite diagnosis. You will need to see an MPN specialist to get the best treatment. As Hunter says ET is cancer with a little c! Hope all goes well for you

[bluebird567 in reply to lizzziep]

Thanks for your reply. Yes I think I'll stand well clear of Dr. Google and wait till my next appointment where hopefully I'll get some answers. Thanks for being so supportive. All the best.

[RedCardRob]

Welcome to the forum.I was informed straight away at an appointment and found I had more questions on the way home.

Although the way you "possible" found out, at least you can make a list beforehand.

Try not worry too much, if it is confirmed you will be given specialist care contacts to call 24/7. So that is a question to start with 🙂

[bluebird567 in reply to RedCardRob]

Thanks for your reply and your welcome onto this forum. If it turns out to be ET I will take on board what hunter5582 said, that it is cancer with a small 'c'. The support you guys give is amazing. Thank you. I hope you keep well on your journey.

[Exeter21]

wait until you get a confirmed diagnosis. I also had similar experience & not a good way to inform patients . I read up lots after blood’s confirmed ET. Platelets were 590 but I was over 60. Also given Hydroxy & offerred no other choice. Body reacted badly on It so I read there was Peg Interferon which I battled to get prescribed. This has been excellent on a monthly dose of 45 . Platelets now 260 & all bloods in range. I did completely review food & eat low inflammatory & no processed foods as MPN suggested. Energy good on injections . But wait until diagnosis confirmed but you have options re drugs .

You can opt to speak with MPN expert . Which I did for treatment advice .

Julia . UK .

[bluebird567 in reply to Exeter21]

Thanks for your reply. Yes I'm going to put off thinking about it until my appointment in October. Such good advice on here. And it's good to know there's more than a singular option and that contacting a MPN specialist is the way to go. Best wishes on your continued good health.

[Mostew in reply to bluebird567]

everyone has offered good advice.

Just want to add, this a an opportunity to do all you can to get yourself in best shape both physically and emotionally.

That way if you do get diagnosed in October you will be able to cope better.

Have you had vit b vit d and ferritin , iron blood tests . ? If not may be good to have done to see if they are optimal….

[bluebird567 in reply to Mostew]

Thank for your reply. I am very grateful for all the responses given on this forum. And grateful too for MPN Voice.

I try to keep active but it has been difficult with the exhaustion. I am feeling more positive about the whole situation now and do as much as I can, which is better for my mental as well as my physical health.

Yes, I have had a FBC just the other day and with more extensive testing so I feel I am being well looked after.

It's just a matter of waiting for my follow-up appointment which gives me plenty of time to digest all the wonderful information given here. Thank you. All the best.