ET triple negative

[FernBee]

Hi. Diagnosed at 16 and I'm now 45.

I've had various medications throughout, some ok, some not so great. They've either made me miserable or become tolerated.

Just wondered if there are any other triple negatives about too.

[Cat1001954]

hi I’m triple negative ET

[Grum1]

Yes I am also triple negative. Diagnosed 4 years ago at 59. I just wish there was more information on triple negative ET available

[Dojojojo]

Hi I am triple negative too. Diagnosed at 56 and now 60 years young. Platelets at 680 at present. Haematologist advised the rule of thumb was prescribing Hydrea when 60th birthday was reached. With the help of MPN Voice contributors I had the confidence to say no without a personalised plan in place as there seems little info about it. Some experts say no need to start chemo drugs yet if there are no symptoms, which there are not.My Haematologist has been great and has now referred me to Claire Harrison in London.

All the best to everyone

[beckyluck]

Hi FernBee,

I am triple negative, diagnosed in 2017 aged 39 after lots of tests, was on Hydroxycarbamide but now on Interferon and tolerating it quite well, only minimal side effects.

Not sure how many of us are out there but I’m sure there are a few

Have a good day

[FernBee in reply to beckyluck]

Hi, I was on interferon on the begining. Got fed up with the side effects. Also been on hudroxi, anegrelide and now on ropeginterferon

[gdpone in reply to FernBee]

Curious so with I’m assuming pegasys you had side effects but not with reopeg (besremi)? I’ve heard it’s supposed to be easier but haven’t heard of first hand accounts of that.

[FernBee in reply to gdpone]

I was miserable. Tired, felt sick, quality of life took a dip. The Besremi seems much better, been on this for 3 months. I take on an evening before bed. Some tiredness and tummy issues around injection day. Otherwise, so far so good

[gdpone in reply to FernBee]

Very glad to hear for you personally and reassurance to me as I’m about to start it soon.

[woozlebop]

Hi FernBee. I’m triple negative and asymptomatic with platelets (last month) at 1100. I take low dose aspirin. I’m 76 diagnosed in 2018. I have blood tests and see my hemo monthly. I recently found an educator in ET (she has JAK2) and medical marijuana. Her results prompted my hemo to tell me to try it before he prescribed HU

My only risk factor is age and I’m sure some will be horrified at my platelet count. However I feel happy to try it and wonder if anyone else has ?

Take care everyone!

[FernBee in reply to woozlebop]

Thank you for replying. At worst my platelets were over 2000. Are there side affects to the medical marijuana?

[woozlebop in reply to FernBee]

Mine does not contain THC - the psychoactive stuff - although the person I’m in contact with has THC included in her CBD (in America). I’ve only just started mine so don’t know if it will do anything for my platelets. I do feel less stressed and sleep well. CBD is an antioxidant and anti inflammatory. There have been no big studies on its efficacy with ET although there are some studies about it with leukemic issues. There are many different strengths and types and I did LOTS of research. Anyway from what I’ve read so far the only side effects from the no-THC CBD are good ones. My hemo and I thought it was worth a try. Good luck with whatever you decide.

[Mostew in reply to woozlebop]

interesting you are trying CBD .

IVE been using a good German one for a few years.

Hasn’t effected platlets, but m eant to be good to help with side effects from Hydroxy.

[woozlebop in reply to Mostew]

My reason for trying was that I and my contact in America wanted to try alternative therapies first.. Her platelets had slowly reached 1000, she started cbd, WITH the THC in it, and they equally slowly came down to 350 (her count last month) She also takes other anti inflammatory supplements like NAC, turmeric, etc. Am I allowed to put her website here? She has a lot of links to empirical research.

[Manaaki in reply to woozlebop]

Hi Woozlebop! I am very interested in that website as I am searching for alternative meds/supplements that may help in reducing my high platelets. Would you pls share that lady’s website with me? Thank You!

[woozlebop in reply to Manaaki]

Hi Manaaki. Yes of course. It’s jessicaharshbarger.com. She’s also on FB running a group called Essential thrombocythemia alternative treatments discussion group. You can message her there too. Let me know how you get on if you like. Take care. Janet.

[Manaaki in reply to woozlebop]

Thank you!!! ❤️

[Scarlett500]

Hi. Im triple negative ET too and 58. It took ages to get diagnosed, nearly a year. Started on Interferon last year as I also have diabetes so haematologist didnt want to wait till I was over 60. Platelets came down to around 450 in about 6 mths.

[TTA_]

Hi!

6 years ago I would have qualified as triple negative essential thrombocythemia (ET), but as I was diagnosed in 2020, the Next Generation Sequencing test showed a non-canonical MPL mutation that is germline. So in my case, ET is inherited.

I do not know if you can access these tests that are much broader and include plenty of pathogenic mutations that can be responsible for essential thrombocythemia, but it would be helpful to know what mutation you have. It could inform future treatment.

These are some studies exploring the non-canonical mutations that can be behind a triple negative ET:

pubmed.ncbi.nlm.nih.gov/379...

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

As I have a non-canonical MPL mutation, my focus has been on this, so this is a summary below of potential mutations:

For hereditary ET, germline mutations can be: MPL K39N; MPL R102C, MPL R102P, MPL R106L, MPL R120W, MPL R170H, MPL V501A, MPL S505N and MPL W515R.

onlinelibrary.wiley.com/doi...

ashpublications.org/blood/a...

There are other papers on both germline and somatic mutations that are non-canonical (often found in patients initially considered triple negative) and could be involved in ET and PMF:

Somatic: MPL T119I, MPL S204F, MPL 204P, MPL E230G, MPL Y591D

Germline: MPL V285E, MPL R321W.

pubmed.ncbi.nlm.nih.gov/264...

Canonical MPL mutations are S505N and W515K.

More interestingly, some mutations are seen as activating mutations:

"we showed that L498W is an activating mutation potentiated by H499C and that H499C and H499Y enhance the activity of the canonical S505N mutation. L498W and H499C can activate a truncated TpoR mutant, which lacks the extracellular domain, indicating these mutations act on the transmembrane (TM) cytosolic domain."

pubmed.ncbi.nlm.nih.gov/319...

I hope you get a thorough genetic testing that can inform better treatment.

My platelets have been above the limit since adolescence, but I was diagnosed at age 38. My only treatment for now is baby aspirin, as my doctor thinks there is not much evidence to support the use of cytoreductive therapy in patients with my mutation.

[FernBee in reply to TTA_]

I also had gene sequencing, nothing came up. It often feels like a waiting game, to see what comes up next.

[TTA_ in reply to FernBee]

There are many ways genetic sequencing can miss non-canonical mutations. Let's hope that more and more mutations are discovered and included in genetic testing. I was lucky the place that tested me does research, so their genetic testing was quite extensive, and I was lucky that the mutation I have was being included in genetic tests around the time I was was diagnosed.

[Luthorville]

Forgive my ignorance, but what does triple negative mean?

[FernBee in reply to Luthorville]

Hi, in a lot of cases they identify a mutation causing the high platlet count. JAK2 CARL or MPL

[Olliebunny in reply to Luthorville]

Triple negative means that it is not any of the 3 known gene mutations but probably a mutation that hasn't been identified as yet.

[Luthorville in reply to Olliebunny]

Thanks that makes sense

[Olliebunny]

Hi I am triple negative, I believe I have had this for at least 20 years but was diagnosed 3 years ago. I'm 58. I cannot tolerate aspirin or other blood thinners. I have been on Hydroxy for about 2 months with various side effects. I am very exhausted. I hope you are doing well x

[FernBee in reply to Olliebunny]

Side effects are the worst. The latest for me is Ropeginterferon, so far so good x

[Exeter21 in reply to Olliebunny]

Just on the exhaustion . I was on Hydroxy & totally wiped me out & exhausted with other side effects. I consulted MPN taken off it & exhaustion went. It was the drug causing it with me. Now low dose Peg Interferon monthly no side effects . I am ET Jak2 . 👌

[Olliebunny in reply to Exeter21]

Thank you, it maybe is what I need to be on. I worry about chop and changing. Will have to discuss with my haematologist. X

[FernBee in reply to Olliebunny]

I've tried 5 different meds over a thirty year period. It's trial and error unfortunately. Some react better than .others.