ET and Autoimmune disease

[CalL1981]

Hello! I was diagnosed with ET a month ago following a BMB though it turns out I’ve had highish platelets since 2007. In 2005 I was diagnosed with Hashimoto’s disease and 2017 I was diagnosed with Autoimmune gastritis. I asked my haematologist if this is all related and while she said there was no direct evidence they are all related she did think there was a reasonable chance this was the case. Does anyone else here have similarly diagnosed autoimmune diseases? I am mostly interested if others have autoimmune gastritis as this prevents the use of aspirin and hydroxy as treatment. I’m interested in other people’s experiences if anyone has a similar situation.

[WeylandYutani]

Hello CalL1981,

I have Hashimoto to offer as well, no gastritis so far.

Cheers!

[Mazcd Partner MPNVoice]

hello CalL1981, welcome to our forum, I do hope that you are feeling ok now that you have had your ET diagnosis confirmed, it can be very daunting, so I hope that the information on our website is helping you, mpnvoice.org.uk

Best wishes, Maz

[Aldebaran25]

Hi CalL1981, I have PV, diagnosed in 2019, and a few months later started developing skin bumps that turned out to be skin sarcoidosis (an autoimmune disease). Apparently this is very uncommon in MPN patients, but I feel there must be a connection: healthy for 57 years and then two rare conditions within a few months ???? So far no other sarcoidosis issues (fingers crossed), as it can attack other organs. Not sure how close sarcoid and Hashimoto are , but I read they may be connected. No issues with the stomach or other parts of intestine for me so far.

[hunter5582 in reply to Aldebaran25]

You are correct on both counts I think. Sarcoidosis is not common in MPN patients but there is a link in involvement of the JAK-STAT pathway. It is very intriguing the treatment with ruxolitinib has shown efficacy for sarcoidosis and MPNs.

"Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling is constitutively active in sarcoidosis."

frontiersin.org/articles/10...

erj.ersjournals.com/content...

pubmed.ncbi.nlm.nih.gov/154...

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

[Aldebaran25 in reply to hunter5582]

thanks for this ! I must look more into it and discuss further with my MPN dept. Now on HU which I believe is helping with the dermatitis, but I must keep on my toes with other potential options.

[hunter5582]

It sounds like you have a smart and well informed hematologist. The body is an integrated system and even "unrelated" conditions can be related.

I have had spikes in gastritis related to GERD. Spikes in gastritis are accompanied by reactive thrombocytosis. During one severe spike, my platelet levels hit their highest level ever, almost 1 million. All inflammation and injury causes reactive thombocytosis. This s normal function. It is just different when you have a MPN and already have too any platelets.

It is also a bit of a vicious cycle. MPNs are at the core inflammatory disorders. The dysregulation of the JAK-STAT pathway causes our bodies to produce too many inflammatory cytokines. This is thought to cause many of the symptoms we experience. We are simply more prone to a host of inflammatory disorders. The dysregulation of the JAK-STAT pathway an certainly exacerbate an "unrelated" condition as well.

I think you are correct to consider the gastritis as relevant to your treatment for the MPN. The good news is that there are other options for ET treatment. There are anti-platelet meds that have a more favorable GI side effect profile (e.g. Eliquis). If you need cytoreduction than there are other options for that as well. Pegasys is the other first-line treatment for ET. Since it is an injection it is not processed though the GI tract, though it can have some GI side effects. These are options you can review with your hematology team. Hopefully your team does include a MPN Specialist. This is particularly important when you have a significant cooccurring disorder.

All the best to you.

[CalL1981]

Thanks so much to all of you for your replies and useful information.

[AngelaBasset]

I had had hashimotos and lichen sclerosis prior to being diagnosed with ET. Now my platelets are normal, WBC is high and I dont fit neatly into any box. Hemotologist just says I have an MPN. Orginally, was told JAK2 positive but after BMB, that was not the case, which I guess is good! Somewhat frustrating....

[CalL1981 in reply to AngelaBasset]

Interestingly while my Haematologist did diagnose an MPN she did say it was probable ET rather than definitely ET. I also have high WBC.

[cmc_ufl]

Just out of curiosity, you said you had high-ish platelets since 2007. How high were they back then, and how did they increase over time? What were your counts at your ET diagnosis?

[CalL1981 in reply to cmc_ufl]

Around 470/480 since 2007 but no one investigated it until this year when the platelets reached 700.

[cmc_ufl]

Which mutation do you have (JAK2, CALR, MPL)?

[CalL1981 in reply to cmc_ufl]

Triple Negative

[cmc_ufl in reply to CalL1981]

I am in a similar situation with my platelets (429-462 since 2011), being evaluated as a potential triple negative ET. Did you have CBCs before 2007 that showed your platelets in normal range? The only early record I have for myself is from 2008, where my platelets were 380.

[CalL1981]

No, I don’t have access to blood tests before 2007 so don’t know if it was ever normal. Do you also have autoimmune disease?

[cmc_ufl in reply to CalL1981]

I do not. I have no other abnormalities (besides a tendency to grow benign tumors like lipomas and have a high mole count). Other than that, nothing. All other blood counts are normal.

[CalL1981]

My platelets have hovered around 550/600 the last year or so. They increased slowly from around 400 over the previous few years. The BMB showed increased megakaryocytes in line with ET. I take B12 and folate supplements and haven’t had any injections as this seems to be enough for now (though I find these upset my stomach a little). Folate seems to be consistently lower than B12.