Hi, I have just been given a diagnosis of ET, and have to decide on treatment choice: Inferon or Hydroxycarbamide. I have already been taking aspirin and have high blood pressure. I am a bit in shock, trying to find out what I can. I have had elevated platelets since 2010....It was last year that I was referred to heamotology, saw a haemotologist who said it wasn't anything to worry about. I then found I have to have a hip replacement, and the hip specialist wanted an opinion from haemotology, as my platelets are around 800. When I got an appointment to see haemotology, I thought it would be a case of seeing if I needed to be on blood thinners for my operation, but no, I am told I have ET. I have only had blood tests, and on here am seeing that pretty much everyone has had other tests - ultrasound/biopsy and such. Should I be checking if this diagnosis is right, or pushing for further tests, or should I just trust the diagnosis and get on with whatever treatment??
Thank you so much
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Minu68
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A BMB is the best way of determining exactly what you have but even this is not 100%. The slides can be tricky to interpret and need an expert.
If you get the option, I’d ask for a BMB if possible to clarify whether ET, no ET, Masked PV or very early stage MF. Also it establishes a bench mark for future comparisons.
If you do have ET/MPN then I would advise you to research Pegasys Interferon versus HU. If you search this forum or check my recent posts you will see latest trial results.
The big debate is whether Pegasys is disease modifying - ie can it delay or even reverse progression in a way that HU can’t. My opinion, FWIW, is that, at least for some, it can. I’m taking Pegasys and found it brilliant - no side effects and has reduced my mutant JAK2 from 80% to 13% in 18 months.
HU still the established front line treatment option. Easier to administer and quicker to work.
Nothing other than blood tests so far. The haemotoligist that looking at my history i have had a high platelet count for 10 years, slowly rising to where I am currently. I have seen most people in here seem to have had bmb. Thanks for you response, most helpful!
Paul123456 has been very involved on this site be providing helpful information. He makes sense explaining that you need a definitive work up by a hematologist. Preferably a MPN specialist
Since you’ve had high platelets for so long, I would probably get a second opinion. A BMB would give a more definitive answer as to the degree of fibrosis and if you have any mutations.
Thank you, it's good to have the views of others in the same-ish situation. I wondered whether it was because I had the count for so long that they didn't feel the need to do one. I'm at such an early stage in this journey, I will be asking about this at my next appointment
At your next appointment ask your haematologist how your diagnosis of ET was determined. It’s a reasonable question.
You need to absorb and understand your diagnosis and what it means for you before the next stage of considering treatment options.
You say that you had blood tests. I wonder if any of those tests was for mutational status, ie. Jak2, CalR or MPL. Although a bmb is now a ‘major criteria’ since 2016 for a diagnosis of ET, not all haematologists adhere to the guidelines. For instance, I am Jak2+ and was diagnosed in 2016 with ET. I didn’t have a bmb or any scan. I did have a physical exam to check spleen, liver etc.
It might just be that the haematologist hasn’t explained clearly to you, which is dreadful if that is the case.
Thank you, I am definitely going to ask how they came to my diagnosis. I don't know what tests they have done, so will ask that too. I am learning a lot from this forum and especially from people like you!
Assuming it is confirmed that you do have ET, you need to clarify why treatment is necessary too. Is it to reduce your platelets ‘short term’ for your impending hip surgery, or, is it for the long term. If it is the latter, that should be questioned, as you’re only 51 with a platelet count of 800.
Treatment is indicated for ‘High risk’ patients who are aged 60 or over, or who have had previous thrombotic events, or platelets over 1500, or are severely symptomatic or, have significant additional health conditions. Patients who are over 60 and are not Jak2V617F+ do not necessarily have to start treatment depending on the other above risk factors. So, a lot for your haematologist to clarify.
You don't need a BMB to have a diagnosis but they are useful as a baseline reading for the future. They don't always show exactly which MPN you have either - even if read by experts. I'd ask what blood tests have tested for, if they have tested for genetic mutations & they have come back positive - the most common is Jak 2+ then its really not necessary for a BMB but still useful to have one on record. I'm assuming you haven't had any thrombotic events but sorry I'm making the assumption that you maybe over 60 & that is why they are recommending medication as well as aspirin. However you don't have to have meds just because you are over 60 (if that is the case) but it would be more so of you had history of thrombotic events. Have a look at MPN voice for information which helps look at treatment options. Ask questions & research your options & a second opinion is always useful. Best of luck & health
Thank you, I'm 51 years old. Another reason I'm thinking the treatment is being recommended is because of my impending hip replacement, which will be safer if my platelet count is reduced. I will ask!
My husband had his gall bladder removed last year with a platelet count of 850 (48 years old) so they will do surgery without meds. Ask for the reasonings & if it's seen as short term. They gave extra treatment during surgery & didn't need to have anti clotting injections afterwards. Good luck with your op. I have to say hubby looked like his old self straight out of theatre, I was concerned but he came out looking so healthy. Its amazing what oxygen & a drip can do!!
I went for Interferon due to my age, active lifestyle and generally not worried about needles or what the side effects might have felt like. Found it easy, no side effects (take paracetamol the first few weeks to help with fluey symptoms) and it was quick to take affect and reduce platelets from 1000+ to normal range. I’m ET Jak2+, 43 years old at Dx June 2018.
That's so good to hear! There are a lot of people mentioning how horrid the side effects are..... I used to be fairly active, but my hip has held me back from managing anything other than work the last couple of years. This gives hope that, once my hip is done, the prospect is not that I'll just be feeling unable to do much because of the treatment for ET
I agree with above. I take Peg and it"s been great. I am 56 year old male , cycle to work. Mild side effects to start with easily dealt with by a couple of paracetamol. I inject 90mgs every other week.
Yay, great you also find the treatment manageable! I'm not bothered bt the thought of having to inject myself at least, not like a lot of people, so that's not a barrier
High platelet counts can be caused by a number of different conditions and you need a range of tests - not just blood tests - to exclude other possibilities before concluding you have ET. I would be asking for a referral to see a MPN specialist.
You have to be your own advocate, asks questions and get informed. If you do have ET you need to be seeing a specialist to make sure you are getting the best possible treatment. Look at the MPN Voice website to get an understanding of ET.
Take a friend or family member to your appointment. Brief them before hand with the questions you want to ask. As the patient you can forget what you meant to say and having someone else asking the hard questions can help avoid patient/ doctor conflict.
You should have a biopsy to confirm the diagnosis. I had elevated platelets between 1250 and 1350 when I was diagnosed.
I was started on interferon but it was really messing my system up. I am now on Hydroxycarbamide and I feel Great. After a year of being on Chemo I am stabilising and feel like I can lead a normal life, or close to it anyway.
Just do what is best for you. We are all different and respond to treatment differently. It may be a life sentence, but it could have been a lot worse. I was sure I had cancer when I had ulcerated feet. At one stage I thought I was going to lose them or even my legs, or even worse, so I am thankful to still be here. Life goes on.
Hi there, I have PV Jak2+ . I was diagnosed by blood test alone because of the Jac2+ mutation.
I have been on Hydroxy since day 1 and it has helped me no end. The numbness in my feet is not as severe thank goodness, my platelet count is pretty much normal now but my tiredness and brain fog is still as severe.
You need to ask your Haematologist how he/she come to the diagnosis of ET and ask them what treatment they would recommend.
I hope you don’t go looking at Dr Google because most of the info on there is rubbish and is scaremongering. If you have any questions then please come here and speak to people who are living with the condition. You will find info on the British Cancer Awareness website as well.
Don’t worry yourself I’ll love, just ask yourHaemotologist about any concerns.
Thanks, I am keeping my research to the MPN Voice site and this forum....and then taking my questions to the haemotologist haha! I have had some of the symptoms, night sweats and leg pains, and get dizzy spells too. Live does have to go on, it's a scary diagnosis, but looks like with treatment I can go on for a long time, thank you for your advice!
Hi there . I too have been diagnosed with only blood tests. I have MPL gene. I was diagnosed following a small stroke last year . I was 54 , never smoked, not overweight, active, ( I had been hillwalking the day of my stroke ) ,vegetarian , meditator, very infrequent drinker etc etc. My platelets had been up at 950 and had been high since 2012 or before .They put it down to fact I'd had surgery for breast cancer at that time .But no one told me they d been high even since . I've been on hydroxcycarbomide since last year . Platelets stable and down at 330 -400 or so . Things seem to be stablising , fingers crossed . It's not great but when when I was waiting for results , we were aware it was either this or a an indicator of a more dangerous cancer . So definitely the better option, having been down that other road .Hope you get sorted . 🙏😊
Mine have apparently been high since 2010, and nothing said to me til last year. Went to the GP about leg pain four years ago, got no real answers and didn't pursue it as then I had another health issue that took the focus. They are recommending the Inferon, but also the hydroxycarbomide. Thanks for letting me know your story!
I would suggest bmb. My doctor was happy he had the original bmb and used my second bmb (years later) to compare to the first. Also, used to verify any progression toward MF.
ET can be diagnosed without a BMB. It depends on which diagnostic protocol the doc is following. Different docs have different opinions. I asked the MPN Specialist about it and he said it was unnecessary in my case. ET that had progressed to PV with no evidence of MF. JAK2 positive with a mutant allele burden of 25%. My regular hematologist told me that BMBs were not as reliable as many think and that it was heavily skill dependent - some facilities just do not do a reliable job with them. If you do opt for a BMB (many do think it is a good idea) then be sure to check on the skill level of the provider and the facility you choose. I would not let just anyone/anywhere do a procedure like this. Best go to a hospital/provider that is in fact a MPN specialist.
If you have not already checked out ton of the lists of MPN expert docs, here is one of the better lists pf patient-recommended MPN expert docs mpnforum.com/list-hem/ .
If you do need to go on chemotherapy for cytoreduction, then be sure to carefully evaluate your options. All of the medications have their own risk-benefit profile. I am one of those who is HU-intolerant, so would have to opt for peg-interferon or ruxolitinib. You will hear from others that they tolerate HU with no significant issues. We are each different in how we respond to any of these meds. if you do opt for HU, there are specific precautions you have to take, particularly if you are still sexually active.
Do be sure to talk to your MON specialist about the risk of Acquired von Willebrand Disease. Emerging research is showing that it is more common than previously thought and occurs at platelet levels in the 800ks. Probably not an issues, but this is important to rule out if looking at a major surgery. Your doc may have already done a vWb panel with ptt/aptt -INR.
All the best. BTW - I have recently had three major surgeries and consultation between hematology and the other specialists is VERY important. Most docs know little about MPNs and may not provide appropriate care without the consultation (that happened to me on one of the surgeries).
I am going to check the list you have provided a link for, great. Am hoping my haematologists name will be on there but probably unlikely haha!
they have already said they want to start treatment of either Inferon or hydroxycarbomide, and I am taking aspirin. I will be asking them lots of things at my next appointment.
Do whatever it takes to consult with a MPN expert. The docs works for you, not the other way around. Ask the doc how many MPN patients he/she has treated. What recent training have you had regarding MPNs. What treatment protocol do you follow? How do you individualize MPN treatment based in the patient's profile? If you are not satisfied with the answer, you can find another doc. If the doc does not like the questions, definitely get another doc.
The approach I use is that I have 2 hematologists. My local ongoing hematologist is a terrific doc, but I am only his 6th MPN patient in 20+ years. He takes time with me, listens, answers my questions, respects my right to make decisions. I also have a MPN expert consulting doc, who is about 2 hours away from where I live. The MPN consultant gives me and my ongoing hematologist guidance regarding treatment. The ongoing doc is fine with this - which is another criteria for a good doc. Any doc who will not listen to a colleague with greater expertise is not going to be a doc I permit to provide my care.
It sound like you have some very important decisions to make. Do know that educated and assertive patients receive higher quality care. Passive patients do not. This is a lesson I learned the hard way. I am now overtly directive with all of my medical providers and make clear what my treatment priorities and wishes are. This actually works well. Relationship based on mutual respect are the best way to practice medicine.
Hi - reading your message of 6 months back sounds like everything I was thinking. Diagnosed 2 months back. Is a bit of a shocker but reading other journeys with mpn's is definitely helpful. My next question for the haematologist is re long term problems that arise from hydrea. Let's hope our journeys goes smoothly
Usually a process of elimination. Was any of the bloodwork for genetic testing - mutations. like JAK2 etc.. Very expensive at least here in the US. I know a lot of people have BMB right off, my Dr waited for other symptoms now classified as post ET myleofibrosis. Don"t freak out a lot of people do very well on HU. Did you or are you going to have the hip replacement?
Hi, have spoken to my keyworker, who tells me that I have the JAK2 gene mutation, hence the diagnosis. Still processing my diagnosis, but so grateful for all on this site who have helped me to understand this condition, and its impact. They still say no hip operation until my platelets are below 400.....so a while, as I haven't even started treatment yet! I need the hip doing, I have no cartilage left, so am in pain the whole time.
When I was diagnosed my platelets were 1350 when they are that high they are not always acting normal and there can actually be a risk of uncontrollable bleeding. I was supposed to have laser resurfacing following surgery for skin cancer and my plastic surgeon wouldn't do it till my platelets were under control. HU worked really well and very fast at bringing them under control - a matter of weeks. So maybe you won't have to wait too long.
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