Update and few questions because i have no reliable sources

[Xuzy]

I posted a while ago about being not able to fit in who criteria but given a pv suspicion based on symptoms and counts.I've sought help to see if at all mpn specialist is available here in India but it seems none are available as i changed my hematologist.

I'm 23 male. First notice elevated hb, RBC , hematocrit on 2018

My WBC also go high as 14 before they drop back to 8 or less.

I have classical pv symptoms of red hands , burning feet. Fatigue

Since last october I've been having episodes of itching like red blotches and hives sometimes like insects crawling on skin. I told this to my hematologist who further ruled out other causes of elevated hb and put me on wait and watch and advice phlebotomy.

It didn't improve my itching at all

My question is do any senior pv patient who have not progressed yet have symptoms? Does a symptom free disease indicate least chance of progression?

I keep reading articles which don't have a solid clue abt it because no one knows.

I'm just trying to infer if sudden onset of itching is normal part of the course and if it can predict future events of transforming.

I'm really in a pinch right now, anxious about my health and not getting answers at any end. My biggest concern is always been this progression which no one can tell. Unknown in life is sure scary!

[hunter5582]

I have not seen any evidence that the onset of itching indicates progression of the PV towards MF. Pruritus is just part of having PV. It is not related to RBCs and phlebotomy would not likely have any effect on it. The itching is thought to be related to cytokines/mast cells. There are a variety if things to try to manage the itching. If it is available, Jakafi (ruxolitinib) is the PV-apporaved medication that seems to be the most effective for pruritis.

healthline.com/health/polyc...

news.cancerconnect.com/myel...

mympnteam.com/resources/itc...

[Xuzy in reply to hunter5582]

Thank you for replyingThe medication is too costly and since my diagnosis itself is not certain i doubt my insurance can cover it.

My doc doesn't even want to put me on interferon.

Only phlebotomy and asprin

[hunter5582 in reply to Xuzy]

At age 23, most doctors would recommend phlebotomy and aspirin. The emerging evidence in support of starting interferon therapy in low risk PV patients is not in common practice yet. Suggest staying very focused on symptom control. The pruritis and erhtyromeralga are significant issues that need to be addressed.

Some people find that a higher dose of aspirin helps with the microvascular symptoms like the erythromelalgia. There is some support in the literature for taking low-dose aspirin twice a day. In my own case, it only took 40.5 mg of aspirin to stop the burning toes/feet issue. We are all different in what we need.

Suggest going through all of the suggested interventions for itching to see if you can find something that works. If none of the other interventions work, then you can revisit Pegasys or ruxolitinib (if your insurance will cover). You would likely need to demonstrate the failure of other interventions to get the insurance approval.

Hope you find a viable solution soon.

[LIGEBA]

Hi Xuzy. It's so difficult when you feel you cannot see the "right" dr.s and you don't feel like you are getting your questions answered. To make things worse, because you can't get answers from a qualified physician, resorting to the internet as our only source, can send our minds down all sorts of paths that can sometimes be unhealthy for us.

I also get itchy as do many with PV. I get it on hands/feet/back/neck. It comes and goes with no rhyme or reason although spicy foods sometimes aggravate it more. I find applying cold helps stop it along with skin lotion. Perhaps you can see a dermatologist to see if something stronger can be prescribed to help.

I am not sure how much symptoms play a role in progression. I am not nearly as knowledgeable as so many others on here. I've had and continue to have many symptoms but remain steady with my numbers. I am currently on aspirin and therapeutic phlebotomies. I was getting the phlebotomies every two weeks but just graduated to every three weeks

Would you be willing to share your HB, RBC , HCT, & PLT numbers? How often does the dr. draw your blood? Are you being followed regularly?

I don't know where you are in India but perhaps you can reach out to Dr. Manoj Unni, who seems to specialize in MPN/PV. He may be able to recommend another dr. or maybe he will be willing to consult with you and your local dr.s via online.

Email: [e-mail]

amritahospitals.org/team/dr...

mpnforumlist.wordpress.com/...

[Xuzy in reply to LIGEBA]

Hello LIGEBAI'll work on the mpn specialist Thank you.

I did consult one which showed up in the list but unfortunately a lot of them aren't really the states level research oriented or interested in this field of mpns here.

My opinion coming from visiting 8 hematologists from last 4 years.

As far as counts go my hb is 17.5 to18.2

Hct from 49 to 53

Sometimes my WBC go high like 14 then next day drop back to 8.

I think I've always had elevated WBC and RBC since age 16 when i first checked my counts.

I've had mutation studies for jak 2 mpl calr including NGS for some high risk mutations nothing showed up. Bmb wasn't suggestive of mpn

My doctors have been doing the wait and watch policy with me. They don't employ a strict under 45 hematocrit but around 47 will do. So like I've been maintaining with phlebotomies every two months.

How long did you have pv? Did your symptoms be the reason for consultation?

[LIGEBA in reply to Xuzy]

Xuzy I was officially diagnosed in 2021 but it turns out my issues started 2 years before. I'm prone to kidney stones and had an unusual situation where both ureters became blocked at the same time. I ended up with sepsis and in critical condition in the ICU for a bit. Had 3 surgeries over 3 months to correct the kidney stone issue and that's when I started noticing symptoms. First my calcium level was high along with my PTH (parathyroid) level. This led dr.s down a path of parathyroid tumor but I ended up never getting tested (didn't want to do the test), but the dr.s ran a lot of other tests that led them to a MGUS diagnosis (prelude to Multiple Myeloma). No treatment - just watch. I never returned to myself and felt awful all the time. The most annoying symptom was the extreme fatigue. Prior to all of this I had been a very high energy person who required little sleep. I couldn't sleep. I had extreme bone pain, thigh pain, hip pain, burning feet, muscle cramps, racing heart, breathlessness, stamina issues, brain fog, blurry vision, headaches, itchy hands/feet/back/neck, low oxygen during the night and terrible depression & anxiety.

I started seeing a hematologist for the MGUS but he never caught the PV. None of my dr.s did (and I was seeing many). I finally pulled up all my bloodwork and tests from my ICU stay forward and analyzed them over many months (it took me that long due to all the symptoms I was having). I finally discovered a trend of my RBC, HGB, HCT & Platelets continually rising and never dropping back down. I was able to note the exact date something changed. My WBC was the only one that fluctuated between high and normal (and still does). I brought my research to my hematologist and he ran the appropriate tests which came back JAK-2 positive.

[Xuzy in reply to LIGEBA]

So much of this diagnosis is what we have to constantly get behind doctors to notice right?And the way research is super slow in this field due to lack of funding or thinking mpn is only old age disease where they can live upto their 80s.

I'm fed of being blown off by doctors. I've ran all places to even get these tests done.

I'm hoping you are feeling better now with proper treatment plan after all that you have gone through.

Best wishes

[mhos61]

It would really be in your best interest to consult with a true MPN Specialist. This expertise is vital, particularly as you’re so young.

Some haematologists will claim to have an ‘interest’ in MPNs, which is to be greatly admired, but this doesn’t give them the title ‘MPN Specialist’.

You seem well researched, so you are probably aware that around 95% of PV patients have a clear cut diagnosis due to being positive for the Jak2 V617F mutation. That leaves 5% who are V617F negative. These patients either fall into one of two categories, receiving a diagnosis of ‘idiopathic erythrocytosis’ (no known cause) or are positive for the Jak 2 Exon 12 mutation. I think around 2% of PV patients have this mutation. Have you been tested for this?

Wishing you the best of luck going forward.

sciencedirect.com/science/a...

haematologica.org/article/v...

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

[Xuzy in reply to mhos61]

Hello mhos61I have been checked for the Jak 2 exon 12 mutations too.

Not just exon 12 but 12 to 15 through NGS

I don't know the reliability of the result as it also depends on sensitivity of test and allele burden but consider I'm jak2 negative but have some very conforming findings with polycythemia vera if not typical. Especially symptoms

[Xuzy in reply to mhos61]

As for mpn specialists i live in India and there are none here . So i consult a regular hematologist