I have JAK2 ET and MPN: Anyone else have these symptoms?

Hello. I was diagnosed with ET, CAL-R back in July. It was discovered in a routine CBC to check my cholesterol. My platelet count was off the charts...2800. All the blood tests, bone marrow biopsies, etc. combined with other symptoms and more tests discovered aVWD (Acquired Von Willebrand Disease) as well. Another RARE card to draw. Counter intuitively, I am not at risk for clotting but just the opposite. Strange to think I was diagnosed with a disorder that causes clotting issues yet I am at risk for bleeding out. I am also taking Hydrea (1500mg daily and I weigh 105 pounds) which has lowered my platelets below 1000 but probably won't reach normal range because my wbc have dropped as well. We still haven't quite found the right balance of meds but hopefully soon. Blood draws every two weeks. Fortunately my aVWD is actually acquired vs. genetic and I didn't pass along to my kids. Still not feeling lucky with two rare conditions. I have yet to meet someone with ET and aVWD. Exercise does help...clears my head and gives me a sense of control in a world where I don't have control parts of my own health. Two chronic disorders with lifelong chemo drugs. Always trying to stay positive even when I question how and why.

That is what my oncologist was concerned with, he seems to be under impression that it would/could happen quickly if it is a genetic mutation.
They ordered my test through Invitae. It might be worth looking into. I was told currently I need to do lab draws every 3 months and if I go on Hydroxy then it would be every three weeks. My head is in a constant spin not knowing which way to turn.

Thanks. New to me. My hematologist prescribes hydroxyurea as first-line drug when indicated by IPSET guidelines (driver mutation, age, clot history).

I know that the leukemia-hydroxyurea link has been studied, debated, and argued for a long time. Is new research showing that the TET2 mutation triggers progression to AML when the patient is on hydroxy?

Just thinking about whether it's worth it to ask about more genetic testing at my next visit. I'm 68, insurance would not cover Pegasys, so maybe moot for me.

TET2 is a gene mutation that was found during my bone marrow biopsy. They currently do not want to try Hydroxyurea with me until my genetic testing is completed. If this mutation is genetic the odds of it mutating to leukemia on the Hydroxyurea is very high. I do not have valve issues but I do have a cardiac history, which makes me a bit nervous with this new diagnosis of ET.

Is TET2 a new driver mutation? All I have heard of is JAK2, CALR, and MPL. I have ET-CALR. Ditto on the fatigue, though hydroxyurea helped with that. Hands and feet are often burning hot or ice cold.

However, I am 68 and also have heart valve issues, so it's hard to know the root cause of it all.

I was diagnosed with JAK2 in early 2021. Taking the drug hydrea has put everything (RBC, WBC, and platelets) back in balance. A bone marrow biopsy revealed that i was also experiencing scarring in my bone marrow - it was in overdrive which is conducive to a 1-3 year life span. So the Leukemia & Lymphoma Society <InformationSupport@e.lls.org> put me in contact with an expert at the OU Stephenson Cancer Center.
Currently I'm attending the Oklahoma Cancer Specialists and Research Institute (OCSRI) and in September 2022 started seeing the OU Stephenson Cancer Center in OKC - comfortably, the two organizations communicate with each other on patients so now i have two sets of eyes monitoring my situation.
Basically, i get blood pulled and reported on every 3 months. Everything has been in harmony for 15 months now. There are back door solutions should hydrea not continue to work. I would never have known about this problem had i not routinely have my blood pulled for analysis because of a testosterone issue. Hope this helps.

I also have ET MPN with the TET2 mutation. I find that if I’m sitting or standing for too long I have what I call “start up pain” my joints become very stiff and it takes a minute or so of moving around to lessen the pain and not look like I am 102 instead of 52. I get the numbness and tingling in my hands, feet and legs. My hands and feet also get ice cold & I bruise easily. I was just diagnosed 2 months ago after 2 years of doctoring and testing to find the cause of my all over joint pain. My oncologist says he doesn’t know what is causing those symptoms but working as a CMA for a general surgery dept at a small hospital, these symptoms make my day very difficult. The fatigue is horrendous and I struggle to accomplish much of anything after work when I get home. All my doctor suggests is baby aspirin and Aleve. I take no other meds at all (other than for my blood pressure).

Wow! I’m so happy he’s doing well.

My connection is known via blood tests. I was very healthy, had the tick bite and 3 months later all of my blood tests changed. My platelets soured very high. My blood work prior to the tick bite was completely normal and more importantly, I felt healthy. Thank you for sharing your story.
Kara

My husband had Lymes and Erlichiosis (another tick-borne disease) 12-15 years ago. In 2021 he was diagnosed with Jak2 MPN, or Primary Myelofibrosis.
We often wonder if the two are related. His hematologists can’t confirm this. I don’t know if there have been studies regarding this. In your case, how were they able to confirm the Lymes connection?
I’m happy to say that he has just passed day +100 post BMT and is doing very well. All the best to you as you continue through the process of recovery.

Hi, the Facebook group may be a good place to post because it has a lot of members. I don’t have those symptoms, however I have several family members with chronic Lyme and I suspect I have it as well but too chicken to get tested. Your symptoms could be from ET or Lyme. Lyme manifests in so many different ways. Sounds like you have circulation issue maybe neuropathy which is definitely part of ET. I’m sorry you had to quit your job because of this.