ET / Pre MF

[otisthedog]

Does anyone have ET/possible pre MF with a high platelet count? Any insight on progression? In the process of changing doctors. Thanks for any info.

[lakeview65]

I am in the same situation. I went to my hematologist this week to get my diagnosis and he said ET, but then I asked about my 1+ riticulin fibrosis and he told me I could have ET with fibrosis. We talked some more, he left the office to print off my Asxl1 results-positive for this and then told me I could be prefibrotic MF, but that the treatment is the same. I started hydroxyurea today. I made an appointment with an MPN specialist and hopefully I can get a more firm diagnosis.

Pre-fibrotic MF seems like a much more daunting diagnosis. My platelets were 697 yesterday, highest ever. Everything else is normal, no symptoms.

[otisthedog in reply to lakeview65]

Thanks for the reply. I agree it is scary not knowing for sure. My platelets were 872. Have not been tested for ASXL 1. I will ask about that. Thanks

[Mortysdad in reply to lakeview65]

Hi Lakeview,Just came across your post and was wondering how your ET/ Aslx1 combo has evolved if any over the last 2 years. Exactly what I have, but also Jak2. Thx!

[lakeview65 in reply to Mortysdad]

Good Morning,

Yes, we seem to have the same issues. I also am Jak2 positive. When I was first diagnosed my platelets were 620 or so, no symptoms that I noticed. I started on Hyroxy in September of 2021, 1000 mg/day and now I take 9/500 mg pills a week and my platelets hover around 450 to 500. No issues that are not manageable with the Hydroxy. I have slowed down, I used to play tennis 7 times a week and now if I play 3 I am happy, but them then life and my mental attitude has gotten in the way. I just dont care about some things like I used to before my diagnosis.

I see An MPN Dr. at Moffit when I live in Florida. Askl1 seems to be a big question mark, we talk about it but they just dont seem to know what it means for me. My local Florida Hematologist likes my platelets below 450, in the normal range so I struggle with telling him I dont want to take more Hydroxy just to have a "pretty number" as my MPN Dr. calls it. He does not agreee with the 450 limit.

I am now in NC and will just go to local Hematologist and I sort of self monitor my bloodwork and if I see an issue I would contact Moffit. I still seem fine, no issues, probably just fatigue/or over age? I am 66.

I saw the post about not taking Hydroxy if you have ASKL1, I believe a Mayo Dr. said it would be an issue . The 3 three Dr. I have seen have never said this. I went to Moffit right before I saw that post so I will have to wait to question one of the Florida Dr.'s

My whole life I worried a bit about my fathers family history of heart disease, my father had a transplant at 63. Never did I think about my maternal grandmother with Leukemia and my paternal grandfather with Non-Hodgkins Lymphoma.

I truly wish I had answers, we are both in the same boat. No one can tell I have a blood cancer and I just live my life taking those toxic chemo pills, they are working to keep my blood counts more normal. I also had 1+ fibrosis reported on my BMB. Moffit reread and said it was not significant and I wonder about that also at times. No new tests to report.

Stay well and let me know if anything changes with you and I will do the same.

S

[WRLM]

I’m jak2+ and my bone marrow biopsy report reads it was a hard sample to differentiate. The sample was atypical for ET, so even though he’s leaning that way, he can’t rule out pre-fibrotic myelofibrosis.

My hematologist said we’d treat as though it’s ET, and then promptly went on a 3-month sabbatical. I’m now realizing the prognosis is quite different and maybe I should consider something more than just baby aspirin to try to prevent progression?

Speaking to my GP today in the hopes of getting a referral to an MPN specialist, hoping I can also get a 2nd opinion on the slides from my BMB. I don’t know how much experience my hematologist or the pathologist have with these conditions.

I would really like to have a more specific diagnosis, though I’m starting to realize a good portion of owning this condition will be learning that I can’t control everything (or anything really! 😂)

[Notmyusername in reply to WRLM]

Hi there,

I was diagnosed with pre mf late last year following a bmb with grade 1 fibrosis and other factors. I'm on interferon to manage my platelets which were 1400 but now down to circa 500. Still a way to go, but getting there. My doc who is an mpn specialist originally suspected that I had ET but after the bmb results confirmed the diagnosis of pre mf.

One of the reasons he preferred the interferon treatment is that he said there was "circumstantial" evidence that interferon might reverse a degree of fibrosis or at least slow down / stop additional fibrosis, which for mf is obviously a good outcome. So that's what I'm on, my next bmb is in Dec so I'll get more data then to see how it looks

Hope you manage to get some clarity.