Hello, Recently diagnosed and Specialist wants to follow for a few years till I hit 60 and then begin Besremi or Interferon (Ropeg). Hunter5582 please weigh in. Last time my platelets WNL was 2012. Slow climb to above 800. Does anyone have specifically ET with Jak2 and Tet2. My specialist skimmed over the Tet2. I have read one study suggesting the additional Tet2 suggests poorer outcomes. I have learned so much here. Any information is greatly appreciated. Thanks for your time and sharing your knowledge. I am on one aspirin daily. Specialist in MPN’s at Moffitt in Tampa. Diagnosed with lab and BMB MD Anderson Houston. Houston wanted my to take Hydroxurea. Moffitt said don’t. I lived in Houston 30 plus years, now stay mostly Florida. Not sure how my Blue Cross insurance will handle this when time comes.
Not finding much at all on Tet2. Thanks everyone. So glad I have this group!
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Will let others chime in - but similar story. ET Jak2+, 53, was on only aspirin from about 2014 - 2021. Platelets slow climb from WNL to about 900/950 (about 100 every year for 6 years) . Was on only aspirin given age and no history of thrombotic events. Platelets spiked in early 2021 to 1,400 some bleeding at gums and time for cytoreduction. Started HU, did not tolerate and moved to Peg. Wanted Peg as only medicine currently known which might slow progression and/or reverse disease course. Been on Peg for 10 months now going well (a few liver questions but gettin resolved) - platelets going in right direction all other numbers WNL.
Do you know your allele burden for Jak2 and TET2. I am also TET2 positive but at a very low burden (4%) while Jak2 was fairly high for ET at 40%. Depending on your allele burden, given the fact that you are Jak2+ and TET2+ and your platelets have been steadily increasing, I think it would be worth a discussion with your MPN specialist to assess whether going on Peg (interferon) now might be worth it. Had my platelets stayed more or less steady, I would have not looked to start Peg until around 60 or 65 years of age..but what we know now, if one can tolerate the medicine, has rising platelets and a meaningful allele burden, I personally, would be pushing for Peg to at least be in the consideration set for my treatment plan.
Thank you for reply. I know my Jak2 burden is low. I will inquire about Tet2 burden. Neither specifically mentioned it. My research and others have made me curious. I have not met anyone with both these, so thanks for sharing that we are in similar territory. I will get back to you on allele burden. 🙂
Hello my friend. I am still looking forward to learning to scuba dive. I priced out a cruise to the Caribbean where I can get my dive certification. Hope to go in the Spring. Sounds like a lot of fun!
The standard protocol for ET age<60 is aspirin and monitor. Age 60 (or 65) is the standard age at which you are considered to no be high risk. There is , of course, no sudden transformation that occurs on your 60th birthday. We all age differently. My MPN Specialist told me "65 is the new 35." Note that some docs no longer use age-alone as a defining risk factor, rather looking at each patient's individual profile. You will have to make up your own mind about that.
Like you, I have the JAK2 mutation plus a non-driver mutation (NF1). The NF1 mutation increases my risk of progression. I do consider this relevant to my treatment decision. The interferons are the one treatment option we have for which there is evidence of decreasing risk pf progression. While that research is PV-based, there is an assumption that it would apply to ET as well. I held off starting on Pegasys for a while, awaiting Besremi approval. I am glad i started Pegasys and in hindsight, wish I had not waited as long as I did.
I would suggest thinking through what your treatment goals and risk tolerance are. In terms of treatment goals, how do you weigh symptom control, risk reduction, and reducing risk of progression? What do you want to focus on in making treatment decisions? What are you willing to risk to accomplish your treatment goals? What adverse effects are you willing to risk or experience?
If you are clear about your treatment goals and risk tolerance, your MPN care team can better tailor your treatment plan to meet your preferences. It will help if you know a bit more about the TET2 gene and the implications of the mutation. Here is a bit of information on this. There is more in the literature.
Regarding getting your insurance to pay for one of the interferons, chances are that it will be lot easier to get Pegasys approved. It is about 1/3 the cost of Besremi and Pegasys has been in common use off-label to treat ET for decades. Besremi is only FDA-approved for PV. Besremi is in clinical trial for ET if you are interested. I had no problem getting Pegasys approved. I had to mount an extensive appeal campaign to get Besremi approved.
Hope the above information helps. Please do be sure to review what you learn with your MPN care team at Moffitt. They are the best equipped to help you acheive your treatment goals.
I am truly impressed with all the articles you found and shared. I am planning on trying to understand more of this information so I may ask appropriate questions. I feel as if most of this is a foreign language. I grew up with School House Rock and so badly need to see this in a School House Rock format. 😂. Your willingness to share your time and thoughtfulness are wonderfully amazing to me. I am always happy to help with Diving. I don’t know where you live, but recommend a dive course near your home. A quick and dirty on a cruise is fine, but tip well and stay close to your Dive Master. The course is all about safety so bad things don’t happen. The more prepared you are, the more fun you have and better chance of handling things that can come up. Diving is the most amazing experience to me, I wish everyone had the opportunity. I am sure we would all be better stewards of our planet if we all dove. Hope to hear of your diving adventures soon! Thanks again, I know helping so much must be a burden, thanks for being a Leader!
I am glad that you found the articles a good starting point You are right that this is like speaking a foreign language. It is a language we must learn to speak in order to understand MPNs and work effectively with our care teams. I frequently have to do secondary reading to understand the articles I review. I have also attended training forums both for patients and for professionals. The webinar by Jyoti Nangalia is one of the better presentations. She has done other presentations that are quite good. She is one of the leading MPN molecular biology researchers in the world.
If you want to broaden your understanding of MPNs, another good place to start is to understand the basics of hematopoiesis. There is an excellent series of seminars designed for medical students that cover the basics in an easy-to-understand fashion. Noting that "easy" is a relative term. These are the Ninja Nerd series.
As you developed the basic vocabulary and an understanding of some of the physiology involved, it does all start to make more sense. One of the things I like about answering questions is that it gives me the opportunity to review the things I am learning. It helps to reconsider things from different perspectives as I learn more. That is one of the best things about this forum - The chance to learn from each other.
Thanks for the tips on diving. The cruise my travel agent found involved doing classroom work before you get on the cruise. The you go on the the practical once on the cruise. This is one of those things on my bucket list. i am really looking forward to it. I once went snorkeling in the John Pennekamp Coral reef Park. It was very cool. Even better to spend more time underwater.
Moffitt and MD Anderson are some of the leading centers in the treatment of MPN's in this country, you are lucky to have access to them. I would suggest you gather all your information and test results, read as much as you can about mpn's (including on this site) and then have serious discussions with whichever hematologist you decide to treat you. I think we have to trust someone with our care, not an easy decision, but I think it is a necessary one. Best to you going forward.
I checked out the helpful links Hunter provided. A good opportunity to refresh the subject for everyone. The 1st two focus on TET2. But it seems TET2 studies are esp focused on PV.
The 1st article has this reference re MPNs:
TET2 is not bad for PV, PMF (ET not addressed) : "The presence of mutant TET2 did not affect survival, leukemic transformation or thrombosis in either PV or PMF" ... "and (TET2s) hold limited prognostic relevance. (But apparently is relevant for IFN, see below)
The ref is 2009 vintage, about when TET2 for blood disease was 1st described, so there should be newer info, but so far this report is holding.
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In the 2nd ref:
On how common TET2 is for various blood conditions, (MPN=7-13%):
Acute myeloid leukemia 12–24%
Chronic myelomonocytic leukemia 20–40%
Myelodysplastic syndromes 19–26%
Myeloproliferative neoplasms 7–13%
Systemic mastocytosis 29%
They repeat the encouraging info from the 2009 study indicating it's the best word right now:"According to Tefferi et al., there were no clinically significant alterations in the prognosis or overall survival rate in patients diagnosed with MPNs based on findings from large cohort studies"
But for PV related CMML and AML: "On the other hand, studies have demonstrated poorer outcomes in hematologic malignancies including CMML and AML associated with TET2 mutations with limited data on PV"
Which order you acquired each of Jak2 and TET2 matters "Patients with initial JAK2 mutation demonstrated a higher risk of thrombosis compared to a more indolent course in patients with initial PV-associated TET2 mutation (Jak2 came later)"
IFN does not reduce TET2, and having any extra mutation such as TET2 can reduce IFN response:
"With regard to the treatment, studies have shown that management with peginterferon alfa-2a can reduce JAK2V617F clones but not the TET2 mutant ones. ... Another study has shown that peginterferon alfa-2a-treated patients with both JAK2 and TET2 mutations had a less significant reduction in the burden of JAK2V617F compared to those with JAK2 but without TET2 mutations. The former group possessed a higher burden of JAK2V617F mutation at the beginning of the therapy- (Solyesh in this thread matches this part but you report a low burden). Furthermore, the same study revealed that patients without complete remission were more likely to have additional mutations apart from JAK"
As usual in our business in this report has many qualifiers.
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There are apparently several types of TET2 mutations, I can't find their significance but could be worth knowing which you have for future reference. Possibly MPN has only one of these types.
Good Morning. My Jak2 is 14%. No mention of Tet2 allele burden. I only had CBC’s, iron studies, and basic chemistry labs until July 29th when I had the visit at MD Anderson. That day extensive lab and BM Aspiration with Biopsy. So no way to know what can first chicken or the egg concerning Jak2-Tet2. I will message MD Anderson about the Tet2 to see they can review slides and report allele burden with Tet2. I did go back to report and it is reported exact type and where mutants occur. I can share if you like. I don’t have enough retained information to help me with significance. Thanks for sharing information and your time. Each time I speak with people from this group, I grow stronger in my understanding. 😊
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