just had my blood test come back as jak 2 positive which i paid for private as my hb was 19.8 on latest ahn i think hct 0.58 but my gp wouildnt order the test as he was blaming smoking and saying it was secondary i have had heart attack at age 47 now 51
think next steps is take this test to my gp for a referral if he will do it
Regarding your question about next steps, the next step is to get a formal confirmation of the PV diagnosis, which may involve a bone marrow biopsy. As I expect you know at this point, there is a big difference in consulting with a MPN Specialist. Most doctors, including many hematologists, have little experience with MPNs. This is the list many of us use to find a MPN Specialist, but it is not a complete listing. mpnforum.com/tsr-the-list/ Others on the forum can speak to MPN-expert docs in your area who are not on this list. The doc you saw privately may be able to recommend someone in the NHS as well if that is your wish
Note - I have two hematologist, one of whom is a MPN Specialist. My wonderful local hematologist, who is not by his own definition a MPN Specialist, handles my ongoing care. The MPN Specialist works with me to determine my care plan. This way I always get two opinions on every decision by doctors who I trust.
The other next step is to stop smoking if you have not already done so. You cannot afford the exposure to carcinogens nor to the hypoxia that smoking induces, causing reactive erythrocytosis. It is actively making the disease worse.
The next step will be to determine an appropriate treatment plan. You will need formal confirmation of the diagnosis to determine a treatment plan. For lower risk PV, the recommendation would likely be to start with aspirin + venesection for someone age 51. Given your prior heart attack and a family history of PV with stroke, you may not be considered low-risk. Cytoreduction may be indicated. Review of options for cytoreduction needs to be done with a MPN Specialist. You can also learn a bit more about your options from a variety of sources, including the MPN Voice website. mpnvoice.org.uk/about-mpns/.... The NCCN Guidelines are another excellent source of information nccn.org/patients/guideline... .
You clearly made the right decision to proceed with testing for the JAK2 mutation. You will need to continue to advocate for yourself and the care you wish to receive moving forward. Wishing you success doing so.
Thank you very much hunter if I need to ask any questions I will ask kindly if I can ask you as you seem to be knowledgeable if you don’t mind I’m so glad I went private at least I can go back to go and said I told you so at the moment I’m having some bone Bain feels like back pain but a dull ache that’s always there
Was there any additional info in the pdf they provided? In particular the % mutation or allelic burden. If not that is one of the worthy next steps. Your GP should be open to ordering this now or at least offer the referral Hunter notes.
Your Dr could be both right and wrong: 2dary polycythemia is possible with smoking, but PV is also very likely with the Jak2+ result. So it's not impossible you have both, a haem or MPN specialist is in order here.
Your platelets were normal so ET plus 2ndary polycythemia is not likely.
I know this seems a daft question but should I now start going on a diet I know I have to give up smoking 100% I was hoping it was negative but at least now I can call the gp incompetent and make a massive complaint imagine if I hadn’t paid for the test I could of had a stroke
The old nag of "eat better and exercise etc" is often offered by those who need to heed it. But it's still good advice and never too late. My other disease has cut my good habits but I still try.
Esp if stroke is a worry, you have direct control of some of its best known risks. Agree its time your GP helps you with the additional MPN risks too.
I’m going to ask to be referred to guys in London I’m in midlands but don’t mind going there as what I read best in country
With Guys you will not need to stress about what best to do next. Best case they will then coordinate with a local haem for your regular Dr visits.
I would like to have their care here in my town (Los Angeles area)
Well I only want to go where best is cos my experiences so far have been somewhat diabolical from my own gp I will be making a formal complaint to the surgery practice manager imagine if I just accepted there take I could be dead as I’ve heard pv is fatal if untreated I’m not overreacting I think the care as been poor I’ve had high blood counts for a cpl of years now I always put this down to the heart damage I unfortunately got whilst waiting 24 hours for a cath lab when had my heart attack the nhs is on its knees
just going to add the result here
Glad you have a bit more clarity. While bittersweet to find out, as you say you now have important additional info. to get help to protect yourself.
Your report seems to say it was run by the Berkshire and Surrey Pathology service.. I googled to see what they said about their testing; link below. I think given that they reported your result as positive it means the Jak2 allele burden is at least 5%.
berkshireandsurreypathology...
Lots for you to digest; ask here and plenty of people will fill in the gaps where they can. Hope you get a quick referral.
Oh sorry, I’d mis-read the categories ( not clear if above or below 5%). Let us know how you get on with the next steps; hopefully it’ll all get properly investigated quickly now.
I was diagnosed with PV from having a Jak2+ blood test. I was told no need for a bone biopsy which I would have to have done if the blood test was negative because of my high red blood cell count with 3 DVT’s.
Well I had to go private for the jak2 test as my gp fobbed me off saying was secondary I’m gunna have a fit at him when I have my appointment
Don't forget you want your GP to do what you ask . Be firm , but calm . They have to refer your to a hospital which will carry out all necessary testsAsk for referral to Mpn specialist to
Not easy , let of steam AFTER appointment here !!!
Good luck
he’s done a urgently referal to queen elizabeth hospital Birmingham and I will ask at this appointment for referal to guys as gp said he can’t do a referal to guys as to be done via where I have my first appointment
Hurray for the referral. Things are on the right track for you now!
Well done. Must be different in various parts of country. My GP was happy to refer me.
Hope he isn't fobbing you off!
he asked if I can print my results and take them to him and what made me laugh he said he didn’t know about this disease I was like are you serious lol
That is not at all a surprise. Quote from one of my docs "What is polycythemia Vera? It has been a long time since I was in medical school." That doc is a great doc! He acknowledged his ignorance and listen to me when I explained what PV is and how it was relevant.
Just a quick question should I ask the non specialist to go on interferon as I have noticed lots of bad things with hydrea from what I’ve read
The interferons and hydroxyurea are both recognized as first-line cytoreductive treatment options. We each respond differently to these medications. Some tolerate and benefit from one and not the other. I am one who responded poorly to hydroxyurea. It was ineffective and I could not tolerate it. I have responded much better to the interferons. My response, however, does not predict yours.
It will be important for you to educate yourself about each of your treatment options. The decision should be based on your treatment goals and risk tolerance. Knowing what you wish to achieve through treatment and what risks you are willing to take to achieve your goals is essential to making a good decision. All of our treatment options come with potential adverse effects. The treatment options work in different ways and can have somewhat different benefits. Both HU and IFNs can be effective for cytoreduction. HU usually acts faster than the IFNs. The IFNs may be more effective in the long run in regards to preventing the progression of the disease. Please note that this a an incomplete and oversimplified assessment of these medications. I would encourage you to take the time to educate yourself about each of your options.
The short answer to your question is that you should be discussing your treatment plan with a hematologist soon. Ideally, this will be a MPN Specialist. Given your profile, cytoreductive medication may be recommended. Note that the IFNs are much more expensive than HU, with Besremi being one of the most expensive medications on the market for MPNs. This can affect access to it.
Wishing you all the best.
Still waiting for referral and been told I should prob be waiting a month this is ridiculous I feel
Hi hunter just wanted to ask a quick questions on my wbc says basophils are very high 0.16 could this be a problem if it’s a stupid question I apologise in advance
Basophils are part of your body's immune system. Basophilia can happen for a number of reasons. One of them is the JAK2 mutation can cause overproduction just like it does for other types of blood cells. Basophilia can also be a reaction to some form of infection, an allergic reaction, inflammation, and more. When not on cytoreduction, I am always basophilic. It has never been considered a big deal. It is just part of how my MPN presents.
I would not want to guess about what your basophil level indicates. That is a question for a MPN Specialist to answer based on your profile. I would not worry too much about it but definitely would seek an answer to what is going on and how this fits into your MPN profile.
Thanks my appointment looks like a regular healthiest appointment will ask for him to refer me to a MPN specialist
Had my appointment and he was very knowledgeable to be quite honest booked in for bone marrow biopsy and a test for alle burden what ever that is and need venesection twice a week
Glad to hear you found a doc familiar with MPNs. Mutant Allele Burden is AKA Variant Allele Frequency (VAF). This is the percentage of hematopoietic stem cells that carry the JAK2 mutation. Not all of them do. Some are wild type (normal). VAF is considered relevant to symptom burden and disease status. It is important to understand that this is not a purely linear relationship. There are other factors that come into play, including non-driver mutations. Broadly speaking, people with ET tend to have the lowest VAF, people with MF the highest, and people with PV somewhere in the middle.
There is plenty of literature available on this topic if you care to look. Here is one of the more recent articles. ashpublications.org/blood/a...
This webinar on MPN Molecular Biology is very helpful as well.
Does a BMB hurt I’m absolutely crappin it lol
I have never had a BMB. Reports on the pain level vary widely. Those who have had one can speak best to the experience. If I ever need one, I will opt for the highest level of pain management available.
Finally got a appointment Friday 13th of this month unlucky for some one that’s my I’ve noticed by basiphil count is 0.17 is this something to be concerned by
Good to hear you are being seen soon. Hope it goes well, despite the date!
Yeah about time but I’m worried about BMB I’m crapping it
maybe have a search on previous posts as I think there has been discussion re. how to make this as comfortable as possible. (I’ve not had one). Good luck!
Well I have had it and that’s the worst pain I’ve ever experienced and still in agony
Very sorry you are in pain from BMB. I was 72 at the time of first one done with local and I do agree that the pain of extracting the marrow and bone is the worst pain I have ever felt but it only lasted maybe 3-4 seconds per sample. Had 5 samples taken as 2 extra due to not enough sample taken in first tries. Doctor was looking at each sample real time so there was no delay. Afterwards, I did have a bit of sore spot at the entry point in my hip but it didn't last long and I was walking well and drove home. In preparation, I found reference to caution if the drill interferes with a nerve that it can cause lasting pain until the nerve settles down again. Warm compress and tylenol pain reliever may help. Best wishes to you.
I’m having to use a stick and I’m 51 lol I’m in agony take away tonight I cannot face cooking in my dads carer
Sorry to read that! Hope it quickly stops being painful.
Hi threre hope u had a good Xmas I’ve had my blood test results come back from my first appointment and I’m a bit concerned my hb was 19.2 and my white blood cell total count is 27.98 should I be concerned I don’t have a appointment now till February and even that’s a telephone appointment should I’ll I be worried with whites that high or is this normal in PV my platelets count is 380
Sorry - I don’t know much about raised WBC (my PV affects my haematocrit RBC and platelets more than other counts).
it’s almost doubled in 2 months and everywhere I read it’s associated with thrombotic events in PV
High WBC is a negative prognostic for PV in various reports as you have seen. But two months may not be yet sustained enough to fall into those considerations. It seems you and Dr are checking regularly. A related number is of particular interest "neutrophil-to-lymphocyte ratio (NLR)". If your tests have these results you can know this ratio. High is also a negative prognostic. My latest was high, likely related to my other condition.
I think from what I’ve read it’s normally associated with advanced PV unless I misunderstood it very worrying I’m still awaiting the result of the BMB
On my nhs app it only showing the total count I know my nuetophils high and basophils last time at drs in October
Well ive had ultrasound today and sad my spleen is enlarged does this mean disease in advanced stage
Hello. It may depend on how enlarged your spleen is. My haemo said there is variation among different body sizes -bigger people tend to have bigger spleens ( but within limits).
You are more likely to be given cytoreductive therapy (interferon, ruxolitinib or hydroxyurea) as a result of this extra evidence of Polycythaemia putting strain on your body. Lifestyle changes also help, so while you’re in this stressful bit of waiting for your appt it’s the usual advice - try to eat healthily, stay hydrated, get gentle exercise and also enough sleep, not drink too much alcohol and if you’re still smoking try to get that down as much as you can. But also try not to stress yourself out by trying to be perfect!
I’m really sorry you’re having such a wait to get expert medical help - but perhaps as the evidence (results) come in you will inch up the priority list.
As RoundTheWorld says here, the particulars of your spleen result are important. Did you get the specific size results? If your Dr recommends treatment of your spleen size, Rux is worth discussing. Rux is known for its potential to reduce spleen.
No did not exact size just was told I had enlarged spleen as I asked if I was ok to fly as I’m off to Egypt for a week tomorrow
Just had my appointment and my diagnosis is p vera with ET Overlap with a aloe burden of 29.9% I’ve opted to go on interferon and I should get a call back tomorrow to go for my first injection
Glad you’re starting treatment. Are they planning to do venesection? (can be a faster way to reduce hct as interferon can take a while to make an impact).
Well done for getting the diagnosis properly worked out - you may have a combination of things going on but now the MPN is known, so risks can be reduced. I know it has been a long road!
I’ve had 4 maybe 5 venesection and my hct is now 0.44 now
yeh with no thanks to my gp heamo not happy with him for not requesting test said it’s disgusting and this doctor seems to know his stuff
Well I presumed I was going on besremi but no they have put me on Pegasus 45mg to start with and then they said they will titrate to 90mg thought there was a shortage of this drug I’m concerned starting this and then they run out lol
I think you need to cross that bridge if and when that happens. I think the bigger centres have a better supply. Where I go big London hospital they are getting 50% of there usual supply but having moved most of us to 90ug rather then 45 they seem to be mostly ok and are hoping the new factory will be running in September.
Ok thanks that’s ok then as my hospital is Solihull Heamotolgy and chemo unit they have also said I have PV with ET overlap even tho my platelets have always been normal just the reds and whites high with alle burden of 29.9%
I'm not sure about "going on a diet" - that's hard work and adds an extra burden in addition to your heath worries. However, recent science highly recommends the "Mediterranean diet", which is not a restrictive diet.
Here is one example:- ashpublications.org/blood/a...
It concludes: "These data suggest that a Mediterranean diet, which is rich in anti-inflammatory foods, may be particularly beneficial to manage symptom burden in MPN. We are now moving on to larger randomized studies to rigorously test the impact of a MED diet intervention on symptom burden in MPN patients."
You can google a mediterranean diet - basically, it means less animal fat and protein, more plant fat and protein (e.g. nuts and seeds), as much variety as possible , and avoiding processed foods. One recommendation is to eat 30 different plants each week (herbs and spices count for this) - this is a fun challenge, as opposed to a restrictive diet. Add things rather than taking things away.
Good luck!
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