ET - how many of you have this condition and then have it develop into MF or another condition

[vickivicki]

Hi all

I'm new here. A little bit about me, I was diagnosed with ET Jak2 positive in 2018 via a routine blood test when I was pregnant with my 2nd child. It wasn't until after I had my baby that the diagnosis really sunk in that this was a condition that I had to live with and maintain. I was initially told to take low dosage aspirin and not at high risk until my count was up around 1700's - they started me on interferon as I was breastfeeding. After a few months, we changed it over to hydroxycarbamide as my symptoms' with interferon weren't great. I started on about 15 tabs thru the week which did nothing then they increased my dose which was great for my platelet count but made me anaemic and really messed with the other blood lines so had to decrease my dose.

Almost 3 years later, I am still on hydroxycarbamide and still figuring the right dosage. My count doesn't seem to want to drop any lower than 600 but the consultants tell me that's okay as long as I don't go over 700.

The point of my post is disease just seems so uncertain - there is no magical number of pills to take, everyone seems different. I was told I was low risk and that I wouldn't need anything but aspirin however I'm now on medication forever. I basically would just like to find out how many people on here were first diagnosed with ET and now it's developed into something else? I've asked my consultant but he, I guess for obvious reasons, is vague.

I'd just like to hear from you and your experiences...if you're out there.

Thanks for reading

[hunter5582]

Glad you posted your concern. It is a common one.

I was diagnosed with ET when I was in my 30s, about 30 years ago. It progressed to PV about 8 years ago. The symptoms have escalated with the progression into PV, but am still managing things well. Currently using PEG-IFN, which works much better for me than hydroxyurea (which I could not tolerate). My current JAK2 mutant allele burden is 26%. It only progressed by 1% in from 2019 to 2020. I am also positive for the NF1 non-driver mutation, which causes another disorder (Neurofibromatosis) and increases my risk of leukemic progression. There is no sign of progression to MF or AML at this point.

The more specific answer to your question depends on a number of variables, including mutant allele burden, the presence of non-driver mutations, the level of chromic inflammation you experience, and more. There is considerable research on this topic if you care to review it. Here is just a bit of what is out there.

patientpower.info/what-is-m...

ashpublications.org/blood/a...

You need to register for this one

practiceupdate.com/C/108101/56

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

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

There are things you can do to reduce your risk of progression. Controlling inflammation is thought to be important in reducing your risk of progression and for reducing risk of developing other cancers. There are diets and some supplements that can help with this. The only disease modifying treatment option we have at this point is PEGylated Interferon. It can, in some cases, reduce mutant allele burden. There is evidence that it may prevent disease progression. The newest form of interferon, Besremi (ropegylated interferon) may be easier to tolerate.

Hope that helps to answer your question.

[Wyebird]

Welcome, we are all different and I felt nothing but hopelessness a few months ago when I continually was feeling tough and getting worse. The result Vit d shortage. My situation is completely different to yours so I hope someone can help you. Unfortunately for me almost 6 years in and my platelets continue to rise. I started on hydroxi then we tried anagrelide. I was unable to do a direct switch as I could only cope with 9 anagrelide a week so it was combined with my hydroxi. So when the opportunity for me to go onto interferon I grabbed it. I started on anagrelide hydroxi and interferon.

After 1 week just dropped the anagrelide completely and some hydroxi.

I do hope you get sorted. You were young to be diagnosed. I was 59

[Pte82]

vickivicki, hunter is right about inflammation control. Also be aware of products containing benzene and toluene as being associated with MPN that may influence progression. My wife, with her oncologist's permission, used 50 mg of red palm tocotrienol twice a day to lower her platelets when neither hydroxycarbamide nor Anagrelide no longer could.

toxictorts.com/benzene-myel...

large.stanford.edu/courses/...

salon.com/2015/06/22/5_toxi...

[Aneliv9 in reply to Pte82]

And she controlled platelets with red palm tocotrienol?

[Pte82 in reply to Aneliv9]

Her platelets remained around 950 after changing from hydroxycarbamide to anagrelide and she asked me to find something to lower them as anagrelide has it's own set of side effects.. The only thing I found was red palm tocotrienol. They gradually came down when she started and the Anagrelide dosage was adjusted down until she came off of it. At that time we believed since the platelets were finally normalized after 24 years it was game over. A year later at Chael Hill the bmb showed progression to myelofibrosis. At that time we learned she was too old for a bone marrow transplant. She was never told about transplants before then or about progression. Aneliv9, it's very important to learn about your options now. More treatments are in trial stages than ever before but a cure is elusive other than a bone marrow transplant. Myelofibrosis is hell.

[Aneliv9 in reply to Pte82]

So she never controlled her platelets with red palm... The progression to MF decreased the levels... Your wife had ET before progression?

[Pte82 in reply to Aneliv9]

The red palm tocotrienol did lower her platelets Aneliv9 as her platelet level was unresponsive to the anagrelide and as I explained we thought that normalization was finally the goal.

[Rachelthepotter]

Dear VickivickiDo you see an MPN specialist? Its worth it. Maybe a telephone consultation. I have MF and was diagnosed about 5 years ago: even in that time there have been new drugs approved. Pegylated ( slow release) interferon plus ruxolitinib has given very good results for PV and MF, ( the Ruxopeg trials) so if your disease does progress then there’s help

Its good that you are being so carefully monitored.

[JaK2ET]

Welcome to the forum!