Anyone with JAK2 neg PV?

[meaboutthis]

Hello,

I'm just wondering if anyone else has JAK2 negative PV. I would love to compare symptoms and treatments. Thanks!

[Aime]

Hi meaboutthis, I was recently rediagnosed with idiopathic erythrocytosis rather than PV due to negative results on jak2 and Xeon 12. It just means I still produce too many red blood cells but they don’t know why. My treatment is still the same at the moment as it was previously. Kindest regards Aime xx😺😺

[meaboutthis in reply to Aime]

Thank you, Aime!

I was diagnosed with this condition about 8 years ago, but think I must have had it for some time before that. I was hoping to compare symptoms... Do you get fatigued easily? For me, it is come and go.

Most days I start off feeling energetic, but by midday that fades and by the early evening I feel finished.

Some days I just feel particularly off, mentally foggy, drained.

On occasion, and I wish I could bottle the mysterious cause, on occasion I feel great, overall, and that lasts the whole day and night. Those times are few and far between and I cannot attach any consistent behavior or lack of behavior as the reason.

I sort of feel like each day just "is what it is," and have determined not to get in my head any more about the bad days. Do you have any of these types of symptoms?

Thanks again,

Jo

[Aime in reply to meaboutthis]

Hi Jo, yes definitely suffer from the fatigue. Brain fog is always worse when I need blood taken off, also headaches and ocular migraines. I think you’re right with your attitude of taking each day as it comes and saying it is, what it is, so adapt and deal with it.

If I start feeling down or sorry for myself, I look at pics of my grandchildren or watch a kids hospital programme and that gives me a kick in the b.... to get going!

Hope you feel better soon. E hugs and kindest regards Aime xx😸😸

[meaboutthis in reply to Aime]

Thank you for the support. Wishing you very well!

[Cali-Med in reply to Aime]

Im new to this but read a lot! Dr Silver from MPN conference says if pV negative look for other diagnosis. It sounds like idiopathic erythrocytosis is just a new name! Do you know any more? My symptoms seem like everyone else ie fatigue, night sweats, head aches. My red blood count 60, hemocrit and hemoglobin both very high monocytes very high! Platelets normal. Hope your doing well!

[Nettie22]

Hi, I'm pv jak2 negative, I suffer from fatigue a lot some days worse than others. Found sometimes I force myself out of bed and just plod around all day , more for my own mind so at the end of the day I don't beat myself up about feeling lazy like I haven't achieved anything. I also have trouble sleeping and suffer hot swears at night recently been getting a lot of headaches, also get pain sometimes in my fingers and toes like someone has stabbed them. The last time I saw consultant which was about 6 months ago he said my symptoms are more in line with ET or MF, see what he says in a couple of weeks when I go back.

Hope this helps a bit and it makes sense it is early lol

Nettie x

[meaboutthis in reply to Nettie22]

Thanks, Nettie, I appreciate hearing from you. I do feel a sense of relief to hear so many with JAK - neg have symptoms like mine. Thanks again!

[Sarahserena81 in reply to Nettie22]

Nettie22 I suffer the same! I am 38. I would love to talk more about CBC results if you are still on here.

[redumbrella]

Hi Jo, I have been Jak 2 neg for 13+ years now my main symptons are fatigue and Bone pain with some minor Brain fog. For the last 6 months or so I have become somewhat more symptonmatic in that both the fatigue and Bone pain have been getting worse, some days the fatigue can be really bad and the Bone pain tends to be worse at night. A couple of days this week I was able to take quite a long walk ie. about 3k or so , I was quite delighted with this as a few years ago I would walk at least 5k most days. Sadly 1k is my limit nowadays and find this very difficult as I love to walk.

At the moment I am on Asprin and venesection when HCT goes over 45. I have my next visit to Haemo next week and yesterday had my Bloods done in preparation for this, they took 6 samples rather than the usual 2 ?

Like yourself Jo I do try not to let it all get me down and most of the time achieve this.

Wishing you all the very best.

Garry

[meaboutthis in reply to redumbrella]

Thank you, Garry, I can relate to many of your symptoms. Thanks for reaching out. Good luck with your visit!

Jo

[DoubtingT]

I have just found out that contrary to early diagnosis of PV, I in fact have SP, first Haemotologist decided I had PV on the strength of high Hct and low EPO but ignored the trip-neg gene scans. After 3 years of no other symptoms and a dramatic improvement in my bloods after being put on Voltarin 50mg by my GP for Mortons Neuroma I decided to look for a second opinion from an Oncologist and was immediately given a Bmb that has now returned negative. My advice to all triple negative PV's is to insist on a Bmb.

[Aime in reply to DoubtingT]

That’s very interesting. I’ve always wondered about a BMB but was told it wouldn’t really give more answers than I have already. I’m having second thoughts now! Kindest regards Aime xx😸😸

[meaboutthis in reply to DoubtingT]

Hi, I have had BMB, which came back normal, thankfully. They can't find a reason for my blood counts going haywire (only RBC, HCT, and HGB). Sometimes I feel special ; )

[Cali-Med in reply to meaboutthis]

Im going to Hemotologist next month due to same issue as you. Dr Silver said if pv negative look for other diagnosis. I can’t get this out of my head! I’m reading a lot about blood letting causing other problems.

I’m confused! Hope all is well with you!

[Selvamok in reply to DoubtingT]

Please reply in detail about "BMB"

[mark382 in reply to DoubtingT]

Excuse my ignorance but what is SP?. Thanks

[DoubtingT in reply to mark382]

Secondary Polycythemia - Similar condition where you have too many RBC but not caused by genetic mutation, can be controlled if/when the Drs find out the cause ~ often smoking, high altitude, liver problems or 1:1000 other possible causes.

[mark382 in reply to DoubtingT]

Ok. Thanks for explanation. I don't have the mutations, but confirmed i have Primary Polycythaemia.

[DoubtingT in reply to mark382]

So you must have shown positive in your Bone Marrow biopsy ? as there is no other way to diagnose PV...... if not then you are being misdiagnosed.

[meaboutthis in reply to DoubtingT]

Hi, DoubtingT,

So from what I understand, PV proper can diagnosed by either having the JAK mutation or a combination of other various criteria. BMB can be normal and yet you can have another combo - like enlarged spleen plus elevated EPO and/or RBC, HCT etc...

In my case, I don’t fit the classic

WHO guidelines for PV, yet there is absolutely (and after the many doctors, 2nd and 3rd and 4th opinions, tests and retests of tests, bone marrow biopsies, etc etc I can absolutely feel confident in saying absolutely) no explanation for my continual rising RBC and HCT and symptoms of fatigue, headaches, burning feet and hands, red face, breathlessness, and more. So my doctor calls it a “variant” of Primary Polycythema, PV, as there is no secondary cause that can be found for it. Whatever the name, it’s a nuisance but manageable.

[DoubtingT in reply to meaboutthis]

Please confirm, You have non of the known mutations, you have had a Bmb that came back negative, you have high EPO and yet your Doctors/Haemotologist still says you have PV on the strength of your blood numbers and a couple of minor symptoms ? High Hgb/Hct, Spleen and itching are also symptoms of Secondary Polycythemia so how did they differentiate ? I noticed that you said that you had hives about 5 years ago ? My secondary is probably caused by an allergic reaction to Yeast which causes Hives, the breakdown of which produces Mast cells that in turn produce Il2-5 cytokines which can mess up the EPO/JAK/STAT reactions. Finding a cause for SP can be very elusive, PV on the other hand is fairly clear cut so I don't think your Drs have really been trying. I hope your not taking any Chemo ?

[meaboutthis in reply to DoubtingT]

Yikes! No, no chemo! And actually after 4 years of phlebotomy-induced iron deficiency during which I was unable to feel like I had a life, so fatigued and brain fogged, my Drs have put me in a course of iron - at first very high but now just a few times a week- which has made me feel myself again, able to drive and live life more normally again. We also moved my target HCT from 42 to 45.

Now I just have more frequent phlebotomies to keep my counts low enough for comfort and safety.

Thanks for your concern!

[mark382 in reply to DoubtingT]

I'll go with my haematologist's diagnosis, thanks. Had all the scans and tests for Secondary P and all negative. WHO class PV on a list of results. I have no idea what other results my haematologist looked at., but she confirmed PV. She is the professional and I trust her implicitly. Looked after me for over 10 years.

[DoubtingT in reply to mark382]

Good luck, My Haemo got it wrong, cost me three years of my working life !

[Cali-Med in reply to DoubtingT]

Thanks! I’m going to make sure to get bmb! I feel like negative is fake. Dr Silver says look for other diagnoses if pv negative.

[DoubtingT]

Hi Meaboutthis, That is good news similar to mine, I suspect the lethargy is caused by the continual venesections lowering our blood iron (anemia); People who have SP only need to keep their bloods below 50 and NOT the 45 as advised for SP. How was your EPO before they started taking blood, If it was low ie <3 start looking at mechanisms that inappropriately activate EPO; Cobalt, Interlukens 2-6, and there are quite a few others.

[DoubtingT]

correction : for PV

[meaboutthis in reply to DoubtingT]

Hi DoubtingT,

Unfortunately, my doctor never took my EPO before they started venesections.

I was entirely without iron stores in my bones (via BMB) and blood for a few years. It was so debilitating mentally and physically and emotionally, my Dr and I decided to start me on a large dose of iron once a day and it made a huge difference initially. My EPO is low now, but I'm not sure if that is still affected by the past venesections or if it's accurate now that I am not iron deficient.

Except for my RBC, HGB and HCT counts, all of my other numbers came back to normal eventually. I felt energetic and could think again and have whole days full of energy! This lasted for 3 or 4 months.

Sadly, for some unknown reason, the periodic fatigue and brain fog have returned, despite the iron, and I do get emotionally down about it now and then.

Because I am JAK2-negative, my Dr sent my blood for a full genetic analysis, which revealed some mutations, but only one that is remotely connected with MPNs, and that only tangentially. So no clarifying information there either.

Anyway, my life otherwise is fulfilling and meaningful and so I feel lucky. The state of my physical challenges is manageable, the limbo of the "why" of it is irritating. I have to remember to look up and out and at others when I get the emotionally down feeling. That helps me.

[DoubtingT]

So did they diagnose PV from the initial Bmb ?

[meaboutthis in reply to DoubtingT]

No. BMB normal. But continually escalating HCT, HGB and RBC.

In 2012, Before starting venesections my numbers were: RBC5.69(3.90-5.10),

HGB17.0(11.5-15.5) HCT48.3(34.5-45.5) .

This caused terrible headaches, lethargy, difficulty thinking, red face and neck, burning in my hands and feet.

Then the years of bloodletting to get and keep my HCT below 42 and causing me to be intentionally completely iron deficient.

This also caused lethargy, difficulty thinking, burning in my hands and feet and a general feeling of being unwell, most of the time, to the point where routine errands felt like a mountain to climb without seeing any marked path.

Now that I am again taking iron, those counts have been steadily rising again, and I am having venesections every 2 weeks, just to keep my HCT below 45.

The upside is that I feel much healthier, headaches, lethargy and difficulty thinking only when close to HCT 45.

Clearly, we are still figuring out the best balance for me.

[DoubtingT]

So with no real mutations and a negative BMB, is your Dr now saying you have Idiopathic Polycythemia or are they still calling it PV ? As soon as my BMB came back negative my Dr re-diagnosed me with IP (cause yet to be confirmed) and increased my venesection trigger to <50; I am waiting for the results of my latest blood test to see if the anti-inflammatory I am on is still working.

[meaboutthis]

My Dr still calls it PV, but explains he thinks its "a variant." Are your HCT and HGB high as well? Also, my RDW CV and SD have been continuously high, but are slowly coming more toward normal over the last months, I think because of the iron I'm taking.

[Aime]

I think in the future that some other mutations or reasons will come to light for those diagnosed double or triple negative for the PV mutations but still have high hct counts. My symptoms have not changed since my rediagnosed, they are identical to the ones I have had since first being diagnosed with PV in 2012. I still have spells that the fatigue is so bad that I cannot fight it through exercise and end up in a useless heap.

Sorry having a moan as I’m starting to slow down and fleeting headaches are back, so blood test on Friday. Kindest regards Aime xx😺😺

[mark382]

As I've had PV for over 10 years i tend to know when i need a venesection. I know get a venesection when my count is >45. Not sure what other numbers are on blood test as my haematologist looks after me so well, I leave it to her.