Anyone with both GCA and also Polycythemia Vera ?

[Iwillwin123]

Is there anyone on this group who has GCA alongwith the MPN Polycythemia Vera ?

[Applesnpears]

Giant cell arteritis?

[Iwillwin123 in reply to Applesnpears]

Does anyone have here - Giant cell Arteritis plus Polycythemia Vera ?

[Applesnpears]

There is some evidence from a study in Sweden that people with CGA are at a greater risk (around six times) of developing an MPN than the population as a whole.

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

Its not clear why the risk is greater, it may be that inflammation plays a part in both conditions but it could be an artefact of medical investigations and ongoing treatment for one condition revealing the other condition.

[Iwillwin123 in reply to Applesnpears]

In my case the MPN - Polycythemia Vera was diagnosed first and then GCA diagnosed later . But as you say I believe it was a case of investigation of MPN revealing the GCA in my opinion . Or then maybe that the immunosuppressant drug Hydroxyurea allowed the dormant varicella virus in nerve ganglia to become opportunistic and trigger the GCA ( which may have been happening in milder forms in the past ) .

I have had sporadic uveitis, unexplained skin inflammation , schwanomma and other inflammatory episodes so radically over last 10-15 years.

The varicella virus theory is not proved of course but just a hypothesis currently .

[Iwillwin123 in reply to Iwillwin123]

I meant sporadically and not radically

[Applesnpears in reply to Iwillwin123]

How are your platelet counts? Elevated levels might be a factor in the inflammation.

[Iwillwin123 in reply to Applesnpears]

My platelets are now stable - but the GCA really took centre stage over PV since last one year .

[Applesnpears in reply to Iwillwin123]

Stable at what level?

[Iwillwin123 in reply to Applesnpears]

Around 350 at the moment .

[Iwillwin123 in reply to Iwillwin123]

No correction - 340 at the moment .

[Applesnpears in reply to Iwillwin123]

The platelet count is good. Have you tried daily cherry juice to help with the inflammation?

[Iwillwin123 in reply to Applesnpears]

Does cherry juice help to reduce inflammation ? I have never known this before. If it does then am willing to try it.

[Applesnpears in reply to Iwillwin123]

Cherry juice has been used to reduce inflammation for years. Its started to be studied more scientifically.

ncbi.nlm.nih.gov/pubmed/306...

My mum used it very successfully for gout. Great source of vitamin C. Worth a try.

[Iwillwin123 in reply to Applesnpears]

Will give it a try . Why not ? Thankyou for the input.

[hunter5582 in reply to Iwillwin123]

The role of the JAK2 mutation and inflammation is linked to a number of issues. Of note, is that is you have Shwanommatosis, then you may have a double whammy going on. This disorder is a RASopathy, which is related to the RAS-MAPK pathway. This is another kinase system that is downstream from the JAK-STAT pathway. The JAK-STAT and RAS-MAPK pathways interact at multiple levels. Both are involved in tumorigenesis and apoptosis. I have become aware of this due to my own history of Neurofibromatosis Type 1 (another related RASopathy). The NF1 lead to an adult pilocytic astrocytoma (brain tumor) that was discovered and treated last year. This lead me to start learning more about the dysregulation of the RAS-MAPK pathway and what that means in terms of its impact. Since I also have a JAK2+ PV causing dysregulation of the JAK-STAT pathway the answers are rather complex. The docs so not really know how these two kinase-based disorders interact and play out. The database is just too small for there to be much in the way of research on this topic. Maybe we need to create some!

[Iwillwin123 in reply to hunter5582]

This is an amazing insight that you have given ! Of course the database for these conditions is too small I agree. How are you doing after removal of the astrocytoma ? And then one wonders if one must one be more alert about such outcomes and if so then how does one do that ? I mean can there be a set of tests to get done regularly to get alerted about outcomes of the RAS-MAPK pathway I wonder....... Could you kindly send to me the papers you read to learn about the interactions of the two pathways and the outcomes ?

[hunter5582 in reply to Iwillwin123]

I have recovered amazingly well from the craniotomy - resection of pilocytic astrocytoma. Had the surgery - open brain procedure - on June 17. Was discharged from hospital two days later. The amazing team at Johns Hopkins Hospital did a fantastic job with the surgery and my post-surgical care was terrific. The tumor was in my occipital lobe and starting to affect my vision. They were able to get all of the tumor out and only has to leave behind the wall of the tumor (the gliosis) that was next to the optic nerve. Just went in for another follow up MRI last week. No sign of recurrence and my brain has recovered from the surgery.

This event is what started me learning more about the two kinase-based disorders. I have had a very mild variant of NF1. Until the tumor developed, I have had nothing other than café-o-let spots. The NF1 mutation causes a deficiency in neurofibromin. This dysregulates the RAS-MAPK pathway, leading to tumorigenesis (that is the KISS explanation). I am still in the process of trying to understand the implications of having a disorder of both the JAK-STAT and RAS-MAPK (aka Ras-Raf-MEK-ERK) pathways. I have talked to my various docs about this, but there does not appear to be a clear answer to what the implications of having both really means. I can't help but to think that it is significant.

Here are some of the articles/resources I have looked at. Lots of secondary reading needed if you are not a medically-trained professional. I am not, so lots of extra reading involved.

Kinase

en.wikipedia.org/wiki/Kinase

JAK-STAT Pathway

en.wikipedia.org/wiki/JAK-S...

JAK-STAT Pathway

jcs.biologists.org/content/...

JAK-STAT Video

youtube.com/watch?v=8uxTTuc...(Not working as expected?)

JAK-STAT Video

youtube.com/watch?v=DigLio2...(Not working as expected?)

RAS-MAPK (aka MAPK/ERK) Pathway

en.wikipedia.org/wiki/MAPK/...

RAS-MAPK video

youtube.com/watch?v=i1f2Rbo...(Not working as expected?)

JAKs – Cytokines - Disease sciencedirect.com/science/a...

JAK2 Inflammation - Hematopoiesis etc. ncbi.nlm.nih.gov/pmc/articl...

[Iwillwin123 in reply to hunter5582]

Superb reading sources ! Thankyou for these. Will keep me engaged for a month to digest all these, though my wife is a microbiologist and will help me with these . Thanks a ton again and please do keep me posted with anything new you spot wrt the jak2 or then the RAS-MAPK . I, for example , seriously explored the CRISPR -Cas9 gene editing tool and PV . However ,after connecting with the relevant researchers , I understand they are nowhere close to a breakthrough with that. For a few days I fantasised about what a breeze it would be to just snip out the mutant gene and get rid of the PV But never mind - Wewillwin after all ....... That is my motto !

[hunter5582 in reply to Iwillwin123]

Will do. Would appreciate anything you find as well. Having a microbiologist as a wife will help. I consult with my brother, who is a PhD biologist-cancer researcher. I keep telling him to hurry up an cure it already!!

[Heather270240]

Yes I have both. GCA 10 years ago and ET 3 years ago.

[Iwillwin123 in reply to Heather270240]

What medication are you taking to cope with both coexisting problems ? Any specific inputs regarding medication or otherwise , that you may want to share will help me a lot please.

[Heather270240]

I am on 6mg of Pred, Aspirin and Hydroxycarbamide 500 mg daily. Both illnesses have similar symptoms especially the fatigue so you will need to be careful when reducing Pred and thinking you are having an adrenal crises when in fact it is the symptoms of the MPN. I don't know where you live but I'm in Kent and my Haematologist admits she knows very little about GCA. Luckily mine is in remission but still taking a maintenance dose of Pred. I was unfortunate and lost the sight in my left eye because I wasn't diagnosed in time. Good luck. I hope this info helps.