Hi, I was originally diagnosed with ET 18 months ago by my haematologist, I later requested a second opinion from an MPN specialist but realised later I didn't actually see the specialist but his colleague who stated I had PV due to high red blood cell count. My platelets have been between 400 and 600 throughout and my RBC has gone from high to below normal. My symptoms are terrible itching and fatigue but fatigue may be due to my age. However, my haematologist still refers to my condition as ET.
My question is, is it possible to have both conditions or did ET progress to PV?
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JP1952
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I have PV with a bit of ET thrown in as I also had high platelets. Treatment is similar for both. I take Hydroxicarbamide to lower platelets and have a venesection when my heamatocrit goes above .45.Diagnosed 15 years ago.
Hi JP I was diagnosed as ET Jak2 + nearly 2 yrs ago. Age now 79. Platelets around a million. Aspirin caused occasional, but uncontrollable nosebleeds. 2 different haemotologists were only interested in pushing chemo or blood thinners and not interested in what I wanted.Asymptomatic apart from FFF (feebleness fatigue and fragility) I turned down HU as I didn't like the odds and Clopidogrel as it seemed totally contra-indicated.
Flash forward to six weeks ago and I finally got to see an MPN specialist whose first question was "What can I do for you?" Fell in love with her on the spot.
She mentioned I might be heading towards PV as my RBC count is high.
Coming back to your question, (sorry, as always, I digress) I am old enough to have done Latin so can tell you that thrombocythaemia means an excess of platelet cells but poly-(many)-cythaemia is an excess of several blood cells.
So according to my logic, PV must include ET, so if you have PV, you don't stop having ET.
My opinion is that it is the MPN specialist who draws the conclusion, based on the whole range of blood result as to whether we have progressed to the point where we should be treated for PV.
As to your not seeing the senior MPN specialist, on my personal experience thus far, I would rather see a haematologist working in a specialist MPN department than the most senior haematologist in any hospital's general hematology department.
For me, it makes all the difference in the world to have found somebody I feel safe and comfortable with. Somebody I can trust. I hope you can find that, too, JP
Hi DottyDaisy, thank you so much for relating your story, isn't it great when you find you're no on your own with your concerns and worries. Really pleased that you have found a sympathetic specialist, I hope between you those RBCs and high platelets are brought under control. I wish I had been more like you and researched before starting meds but I was given the impression that I was at very high risk of a thrombotic event and I mistakenly thought lowered platelets would stop me itching. But I am where I am now and should be starting Pegasys soon.
I asked the question this morning, ET or PV because I read somewhere that it was important to establish which blood disorder you had, and I don't know which to choose when form filling.
Hi,I think there are some gray areas in differentiating diagnosis between PV and ET. PV is defined by high erythrocytes but can have high platelets and leukocytes. Bone marrow is general different in the two diseases (I believe). see reference.
that being said, treatment may be similar... Treatment should become personalized because even within ET and PV there is alot of variation in individuals with the disease.
Thank you, that's really interesting because I had a BMB but never given any results. I've checked back on my blood test results and I definitely had high red blood cells at this time.
I was diagnosed with ET about 30 years ago. It progressed to PV about 8 years ago. My old hematologist/oncologist missed the progression and still had me diagnosed with ET for five of these years. This became evident when I consulted with a MPN Specialist. When it became clear that it had progressed and he missed it, my old doc fired himself from my case and referred me to a colleague with greater expertise. He said "Your health is more important than my ego."
There can be some confusion between ET and PV symptoms. Some look at this as a continuum of MPNs. Others do not. You will sometimes see refences to "Masked PV" as people can indeed have PV that looks at a certain level more like ET. It is important to keep all of the terms straight when evaluating your status.
Thrombocytosis = too many platelets
Erythrocytosis = to many red blood cells
Leukocytosis = too many white blood cells
Essential Thrombocythemia = thrombocytosis only
Polycythemia Vera = Erythrocytosis that may or may not be accompanied by thrombocytosis and leukocytosis.
What you are describing is consistent with a diagnosis of polycythemia vera as the MPN Specialist suggested. This does matter as it impacts the treatment for the MPN. The highest correlation for risk of thrombosis with PV is actually erythrocytosis (and leukocytosis) rather than thrombocytosis. The treatment protocol is to keep hematocrit below 45% for males and 43% for females. If you are above the age of 60/65 then the doc would in addition to recommending venesection likely recommend cytoreduction. Be sure to evaluate all of your treatment options for your presentation of PV, looking at the risks/benefits of each choice.
Here is a couple of useful references as you start this journey. All the best to you.
HiI was wondering where abouts you are.We are in the UK and have been trying to find an MPN specialist.My husband has pv jak2, and is not really happy with his hematologist.We have asked his gp for a referral to another one, but they say he has to ask his hematologist to refer him.
We feel by doing this his relationship will deteriorate.
Wishing you lots of luck on your journey with the disease.
I was also concerned about upsetting my haematologist by getting a second opinion but am glad I did. I contacted 2 specialists directly by email who both agreed to see me but asked I get a referral from my haematologist. I got this by dealing with my haematologist's secretary, any frostiness I ignored. I chose the nearest specialist due to the pandemic. I only saw him once but he wrote a very long letter to my haematologist with a plan for me to be considered for a rux trial and change of meds. He also gave good advice about dealing with the itch (the haematologist did not believe the itch was due to my blood disorder and sent me back to the dermatologist for endless ointments which exacerbated the problem). I am now back under the care of the haematologist but things are going better.I am in South Wales in the UK. I found a list of MPN specialists on Google. Hope this helps.
Any doctor that would prioritize his/her ego over your health should be replaced. Ethical doctors welcome consultation from a more knowledgeable colleague. Never hesitate to seek expert consultation for a rare disorder. Here is a list of MPN-expert docs. mpnforum.com/list-hem./
My original hematologist diagnosed me with Et jak 2 positive in 2008. Around 2016, he said I had progressed to PV. When I pressed him for an explanation, he said my bone marrow was getting more active and sometimes it looked like Et and other times, PV. To complicate matters, my red blood cells started to drop, but my platelets kept increasing. I finally decided to look for an Mpn specialist in 2018. I brought her my original diagnosis and the last 6 months of bloodwork and she did another BMB and tons of bloodwork, and said I didn’t progress to PV at all but I progressed to MF intermediate 1. The best thing you can do for yourself is to see a specialist. I’m just sorry I waited so long. I hope you get things sorted out soon so you can get the proper treatment.
Hi JP, I am somewhat similar to you. I was diagnosed 3 yrs ago as ET Jak2+. My platelet count has been between 450-620 since then. My red cells, Hct, Hgb have been slowly rising since then until this March when they took a big jump up. My original BMB was read by 2 different pathologists and read out as ET. My mpn specialist said I had ET. My diagnosis has been changed to PV. My MPN specialist now says I likely was always had PV and that many people with PV are initially diagnosed as ET. He said that if diagnosed as both then you are likely PV. He said that the EPO reading which was low back in 2018 should have been the sign that it was really PV although the RBC, Hgb, and Hct were all within normal limits at that time. I think that being Jak2+ and having a low EPO level are enough to diagnosis PV. Almost all PV patients are Jak2+, I think about 50-60% of ET patients are Jak2+. So, my reading is that I was always PV although it may be that I progressed from ET to PV, my specialist said that happens in about 15% of ET patients.I am not a MD and certainly not qualified to tell you whether you have ET or PV or something else, just wanted to share my similar experience with you. I am now taking HU.
Hi Meatloaf 9, thank you so much for that. Can I ask what is EPO?Just for update, my haematologist rang unexpectedly today (appointment next week) to say I didn't make the criteria for mithridite trial because I didn't have PV. I reminded him of what the specialist said and he consulted the letter from the specialist and conceded I did.
Hi again, EPO (Erythropoietin) is a hormone mostly produced by the Kidneys. It stimulates the production of red blood cells. It regulates the concentration of red blood cells and Hgb in your blood. If you are anemic your epo level may be quite high and if you have too many red blood cells your epo is likely low as you don't want to make more rbc's but you do because of the PV disease. It is measured by a simple blood test. You should have it measured if you may have PV. These are my understandings, I am not a MD.
Never heard of mithridite, are you going to participate in the trial? Clinical trials are the way new and better treatments are developed. Good luck with what you decide to do and keep us informed of how things are going as there is always someone on this forum with helpful advice, they have helped me. Best to you.
Thank you for answering my question. EPO levels have never been mentioned to me but the haemo did state that my liver and kidney functions are fine. I can see from the responses from everybody that that to have ET or PV is not a clear divide. Thank you everybody for your input.The mithridate trial is a phase 3 trial of ruxolitnib versus hydrea or Pegasys, it was suggested to me by the specialist I saw last September as a way to get me on rux. He thought rux was my best chance to get rid of the itching. Even if I am accepted there will only be a 50% chance of getting rux and I may have to stay on the hydrea for quite a few years, it also involves more blood tests and BMB.
If I am not accepted on the trial I am going to switch to Pegasys, again it's all about the itch.
Very interesting question and you have received great responses here. I too was diagnosed with ET due to consistently high platelets (for about 5 years before formal diagnosis). But I also had l Leukocytosis (WBC also consistently high) and very low EPO readings. My RBC has always been in normal range - not even close to high. Specialist ordered a BMB and had it read by two specialists - they came back with a diagnosis of ET..given the low EPO and the high WBC, I got another BMB (read by different pathologists) which also confirmed ET (for the time being). My specialist indicated that you can look at MPNs as if on a related continuum or ET as a possible subset of PV - for now they have me classified as ET. I have started cyto-redution and have bloods drawn every 2-3 months. We will continue to monitor and if we see any change in the blood work, another BMB will be done and we will reassess.
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