ET or PV

[doglover49]

I was diagnosed with ET about a year and a half ago I just take an aspirin a day, so I had my 6 monthly telephone call from haematology just before Christmas and they are telling me my haemacrotic levels are high and I have to get a venesection, she mentioned PV to me, just saying she knows I hav been diagnosed with ET but this symptom is more in line with PV, I also have jak 2 mutation, am not entirely sure but does this mean it is PV I have?? And does having a venesection help? I have been saying all along I am still finding the fatigue hard to deal with, also feel quite weak a lot of the times, not much energy etc

[EPguy]

High HCT does bring the possibility of PV. How high makes a difference. Here is the WHO

criteria.

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

Also in this table if your HCT is especially high you don't need a BMB (marrow biopsy) to have Dx of PV:

"Criterion number 2 (BM biopsy) may not be required in cases with sustained absolute erythrocytosis: hemoglobin levels. 18.5 g/dL in men (hematocrit, 55.5%) or 16.5 g/dL in women (hematocrit, 49.5%) if major criterion 3 and the minor criterion are present. "

--

A more certain Dx might require a marrow biopsy, but as seen here, not always.

Jak2 occurs in both ET and PV, but PV is near always Jak2 while ET can have other mutations instead.

-

If your HCT is above 42-43 you likely do need a venesection, esp if your Dx is PV.

WHO MPN criteria

[hunter5582]

There is a difference between ET and PV and how they are treated. Sometimes a BMB is not needed to distinguish as EPguy notes. Suggest seeking a clear diagnosis to better guide your treatment.

Venesection is often the first level of treatment for PV. Venesection controls erythrocytosis by inducing iron deficiency. The body cannot make hemoglobin / red blood cells without iron. Some people are able to tolerate the iron deficiency while others are not. Unfortunately, in the long-term, chronic iron deficiency can have its own side effects. These can include fatigue. This does not mean that one should avoid treating the erythrocytosis. it is more of a risk factor for thrombosis for people with PV than the thrombocytosis.

We are each different in how our MPN presents and in what our treatment needs are. We are each different in how we respond to the treatment options. Each person needs an individualized treatment plan. The plan will invariable change and evolve over time. It is very important to consult with a MPN Specialist to ensure optimal treatment. Just in case you have not seen it, here is a list. mpnforum.com/list-hem./

All the best to you moving forward.

[PhysAssist in reply to hunter5582]

Well said, but I would clarify things a little by saying that phlebotomy/venesection primarily treats the increased risk of thrombosis by lowering the blood viscosity caused by elevated red blood cells counts, for which Hct is really only the best recognized and studied marker, which is why it is used as the goal/target.

There is a goal to lower/slow the rate of erythropoiesis [making new red blood cells] which classically is primarily achieved by inducing Fe-deficiency anemia, but that is a less well-defined and longer term process.

Blood-letting/phlebotomy/venesection makes an immediate difference in your risk of clotting by removing red blood cell mass now.

Please consider taking a serious look at the posts herein by EPguy, Hunter, and lastly and least by me regarding the use of interferon [IFN] to not only treat the life [and overall health]-threatening risk of inappropriate clotting, but disease modification [that is to change the course of the MPN by reducing the ever-present risk of transformation to Myelo-Fibrosis [MF] and/or the much less likely, but much more lethal Acute Myelogenous Leukemia [AML].

Best,

PA

[hunter5582 in reply to PhysAssist]

Nice summary of how phlebotomy works.

[PhysAssist in reply to hunter5582]

Hi Hunter,

Thanks- your explanation of ET vs PV and the MPN variability was well-written as well.

I'm in limbo as far as follow-up until 1/6/23, because my 12/23/22 appointment [at Roswell Park in Buffalo, NY] was cancelled due to the Snow-pocalypse that occurred that day, but I feel great and haven't had any side-effects so far, so I'm hopeful as always.

Best,

PA

[HazeBlue]

I’ve had ET for over 10 years . Only had one venesection and that was this year . I take Hydroxycarbamide and aspirin. These Symptoms are similar . High platelets in ET and high red count in PV . I think these conditions can cross over . Wasn’t pleased to go for venesection and it made my BP drop and felt faint but if they give a saline drip at same time it helps . Just didn’t suit me. My brother goes for venesection every couple of months . Have it if they are saying required . Might not need another. Ask about starting a treatment medicine . I write down any queries I have before my appointments. Good luck with it. Happy new year .

[doglover49]

Thanks for replies, I just wasn't sure if this was something that happens when you have ET, they have said to try it and see if the haemacrotic levels come down, I totally wasn't expecting them to say anything had changed so I didn't really take everything in at the time

[Barbados19]

When I was first diagnosed (JAK2+) in 2018 I was handed leaflets on both ET and PV. I had high platelets and high red blood cells. I have also had a BMB. It was only later reading on this site that people said you can’t usually have both. However, my consultant told me that I have ET blurring with PV. I asked some of the top MPN doctors about this at the Living with MPN’s event I attended in London in November. They confirmed it was something that can happen. I have only had 3 venesections (thank goodness as the experience is horrific for me, it takes about 50 minutes to get any blood out and last time they had to stop at 350ml as I was on the verge of passing out).Having the venesections did bring my haematocrit down temporarily. It’s quite ironic that I have a blood cancer and am needle and blood phobic but am getting much better at handling all the blood tests. Anyway, I have been taking Hydroxycarbamide for a year as I’m now over 60 ( along with daily aspirin) and all blood counts are coming down. I have to say I don’t feel any better. Still fatigued, tingling fingers, tinnitus terrible. I had been told by my MPN specialist that this would be the case though, symptoms of the MPN being the same as the side effects of the Hydroxycarbamide. Generally though I am doing very well. I find going out and talking with friends helps loads. Distracting myself so I don’t focus on the tinnitus too much seems to work. Perhaps you could ask your consultant if you are blurring between ET and PV. Best Wishes, Elizabeth

[PhysAssist in reply to Barbados19]

Hi Elizabeth,

Have you considered looking into an interferon-based treatment?

My [pre-existing] peripheral neuropathies, acid reflux, canker sores, and tinnitus only got worse on HU, and have all either gotten better or gone completely away on Besremi.

Not to mention the new onset of fatigue, balance problems, and brain fog that occurred with my starting HU...

Even though I'm well past the 60 -year-old cut-off they use to deter us from pursuing INF, I'm tolerating it with no side-effects, despite the dire warnings of joint pain, fatigue and crippling depression they used to try to dissuade me from it.

Heck, if it's safe enough to recommend to younger pregnant patients, why wouldn't it be so for us??

Best,

PA

[Hopetohelp]

Do you know what your Haematocrit and red blood cell results are? I have et jak2. Was called in well over a year ago as the red blood cells were rising, although they never went above normal range, and platelets and Haematocrit rising too. Said I couldn’t have venesection as body would think it was under attack and would create more platelets. Platelets were around 800. Started Pegysus and am very happy with it. Now I am recorded as having et, and not pv anymore. Worth asking lots of questions when you get the chance. Ps Haematocrit was at top end of normal. I like to get my results which I ask for and they email them to me so I can make an informed decision myself too with guidance from the experts. Let us know how you get on

[Nikon7]

PV and the JAK2 mutation can cause changes in red blood cells, white blood cells and/or platelets. Venesection helps reduce the burden of too many red blood cells and decrease the cardiovascular risk of clotting but fatigue can follow that procedure as well, due to a reduction in iron. I find shortness of breath with some activities after a venesection so I keep my filtered water uptake high, take a nap and decrease the intensity of my workouts (as needed) after a venesection OR before a venesection, when too many RBC’s can also slow me down. My analogy is that PV leads the dance steps and I follow. I hope you can find the right dance steps for you. 😘

[PhysAssist]

Hi DogLover,

Greeting from a fellow dog lover, who was diagnosed with PV in 6/22.

My experience was somewhat different from yours in that my Hgb and Hct were higher than normal and slowly increasing for at least a decade, despite my going to the Red Cross and donating double units of red blood cells [RBC] at their blood drives as often as they'd let me- at least until COVID.

Then in April of this year, my red blood count [also RBC], was also elevated, which it had never been before, so my PCP sent me to a local Heme/Onc, and in the approximately 2 month's time between the April draw and the one at my first Heme/Onc visit, my WBC's and Platelets had risen to well above the normal levels. In a word, my presentation was of the classic PV picture, except that I hadn't had any unexplainable spontaneous clotting issues. My JAK2 allele frequency also fit in well at 48%.

While your picture may have been slightly less clear because of your platelets being elevated first, once your RBC, Hct, and Hgb also became elevated, it becomes unlikely that you wouldn't eventually meet the criteria for PV. It may also be possible that your dominant mutation is something other than JAK2, and that's why the picture is different, although it's not a given.

May I ask what your Red Blood Cell counts are/have been?

I would also prompt you to ask your care giver about increasing to the 81 mg aspirin twice daily that is the usual recommended dosing to prevent clots.

Best,

PA

[SeaweedSalad]

Hello Doglover49,

I was diagnosed with JAK2+ ET almost 1 yr ago in February 2022, I am 37 deemed low-risk, so on daily baby aspirin and similarly, at the first 6month check-up they said my Hct & RBC was high and actually it looks more like PV, and had my first venesection in Sept. Hearing from others there does seem to be blurry line between ET & PV. I thought maybe sometimes it takes time with blood tests to see/ be sure of the pattern when newly diagnosed. I don’t personally believe medical diagnosis are always an exact/instant science in general. I seem to be mostly asymptomatic thus far, but I was surprised how much better I felt after my venesection. I didn’t even know I had been feeling ‘off’ as such, but it was like my head had been a pressurised dusty table top and I’d just been wiped clean feeling clearer and fresher. It now seems a slow process of determining frequency, my counts were fine after 1month so they said come back end of Jan! I asked about a BMB but was told the risk outweighed benefit as it wouldnt change my treatment options, that it would be more relevant when a step-change in treatment needs/ options occurred. But everyone is different and the age groups seem to have different meanings re:counts/ treatments. Hope it helps to hear another story and do ask for copies of your blood tests so you can keep your own record.

Best wishes,