ET with CmPL anyone??

[Quercus53]

I have the above variation but cannot find anything about it. All is see is JAK2 and CALR. I can't be the only one on this forum, surely??

Any info would be good but I would like to know how life expectancy is affected - hopefully not! - and is there more of a chance of the disease progressing to something much worse - again, hopefully not!!

Thanks everyone

XXXX

[socrates_8]

Gooday Quercus53...

Hope you are doing well...

Sorry, however I am not aware of what "CPML" even is... could you be a tad more specific please?

There may be some others here... (?) However, I've not heard of your condition myself.

Sorry that I could not be of more assistance, I do hope that someone else can...

Very best wishes my friend...

Steve

(Sydney)

[Quercus53 in reply to socrates_8]

Hi Steve, yes exactly!! It is a variant/mutation that my doctor said I have, I thought I was CALR but she confirmed I wasn't in my last tel call. I just assumed I could look it up with Mr Google so didn't ask any more. To be honest she has a very strong accent and I find it a little difficult to understand especially in doctor-speak!

[socrates_8 in reply to Quercus53]

Where are you based Quercus53?

It might be a 'Typo' & mis-communication...

There is a known 'Driver Mutation' called 'MPL'

Perhaps, this is the one she means... Best you try to clarify it again, especially given her strong accent too...

The Three (3) main known 'Driver Mutations' are:

JAK2; CALR Types 1 & 2 & about 50 others... & MPL

All three DRIVERS, activate the JAK-Stat pathway, some of them have a higher Allele Burden (AB) than the others...

However, ET is a largely a long-lasting Chronic condition that certainly can impact our Quality of Life, but only somewhere between 5-10% ever progress to MF... or worse...

So fingers crossed buddy...

Best wishes

Steve

[Quercus53 in reply to socrates_8]

Thanks for your help

[socrates_8 in reply to Quercus53]

Happy to try to assist ...

[Quercus53 in reply to socrates_8]

Sorry Steve, finger trouble CmPL

[Mazcd Partner MPNVoice]

hi Quercus53, is it definitely CPML, and where you told about it on a blood test result? Thanks, Maz

[Quercus53 in reply to Mazcd]

oops sorry CMPL, It was diagnosed from my first appointment and associated blood test

[socrates_8 in reply to Quercus53]

Hmmm...?

Methinks that "C" could be a Typo... Possibly...? Because "MPL" is a known ET mutation, and you did say that you have ET... (?)

Best you double check with your doctor before you concern yourself much further with what it is...

If her accent is difficult, ask her to write in an email, in English please...

Best wishes

Steve

[Quercus53 in reply to socrates_8]

thanks again Steve

[socrates_8 in reply to Quercus53]

Pleasure always...

[Mazcd Partner MPNVoice in reply to Quercus53]

I was wondering like Steve, if it's MPL you are referring to, I definitely think you should check to make sure. Best wishes, Maz

[socrates_8 in reply to Mazcd]

Hey Maz...

Hope ALL's well...

Yes, it does seem a rather interesting acronym, does it not?

Best wishes

Steve

[Mazcd Partner MPNVoice in reply to socrates_8]

Hi Steve, yes thank you all good here with me, hope all ok with you. Maz xx

[Doggy1903]

Good morning . Yes if you are mpl mutation, I am a match with ET and on Hydroxcarbimide. Will be interested to know how you get on and how you are . I was diagnosed nearly 3 years ago then aged 60.

[Quercus53 in reply to Doggy1903]

Yay at last I've found somebody else! I was diagnosed in 2018 and have refused Hydroxy as it is so toxic. I am 67 so classed as high risk but my platelets are steady around 580 so I'm just taking 75mg aspirin daily. I have told my consultant that I will consider slow acting Interferon if the numbers start to rise, she is now resigned to having a rebel for a patient, probably a stupid rebel, but I just don't fancy taking those meds.

I also have RA which is how I was rumbled with ET following regular RA blood tests. Without those I would never have known as I have no symptoms whatsoever

I have had the AZ Covid jab and have another 12 weeks to wait for the second one. Rumour has it that the efficacy of the vaccine is diminished if you have blood cancer, not sure how true that is.

Keep well and thanks for your reply

Jill

[Wyebird]

Hi I just typed in the initials and up came the info. It is my understanding that most people have the MAIN Calr or Jak2. Those are just headings and that Calr in itself has many more categories. Yours is yet another mutation. I suggest you ask for clarification. Are you in the Uk?

[Quercus53 in reply to Wyebird]

Hi, yes I'm in the UK, more mutations to choose from than the dreaded virus! A lot of the hits on the web are meant for the professionals who treat us, pretty hard for the average patient to understand. Thank you xx

[Wyebird in reply to Quercus53]

Yes I agree. Good luck questioning your heamo. If still no luck maybe Maz on MPN voice.org.uk can help. Good luck xxx

[mhos61]

Found this: cMPL mutation (2007) a bit dated, but informative. Basically MPL mutation, don’t know what the ‘small c’ preceding indicates.

ashpublications.org/blood/a...

[Tico in reply to mhos61]

Well done Mary, just had a look and I think you've hit the nail on the head. Tina.xx🤗

[Quercus53 in reply to mhos61]

Wow, thank you!! Now I just need a medical dictionary and a doctorate and I'm away!

[SRH55]

Hi, I have ET with the mpl mutation, I was on hydroxy but now I’m on Pegasus interferon & aspirin. My white blood count drops from time to time, but it’s corrected with a change in the dose.

Best wishes,

Shirley

[Quercus53 in reply to SRH55]

thanks for your reply, stay safe xx

[Nettie22]

Hi, I have PV , MPL, Jak2+, DMNT3a mutations one of the few that has the MPL and Jak2 apparently with PV. on Hydroxy and clopidogrel. Diagnosed in 2017 at 45 years old. Side effects aren't that much different to everyone else my consultant has said I have the best chance of living a full life but keeps a close eye on what's going on.Make sure you try and clarify with your consultant what it is it will help give a bit of piece of mind.

Nettie xx

[Quercus53 in reply to Nettie22]

thanks Nettie I think a full life is what we all want - and a chance to get out of shielding!!

[Cookiebaker]

I have ET with MPL mutation. I am 71 and have taken hydroxyurea since January 2019 .

[Quercus53 in reply to Cookiebaker]

Thanks for your reply, it seems there are a few of us around but from what I can gather, not that many. Hope you're keeping well on your meds. xx

[musicworks]

Hi all, this is my first post. I was diagnosed in 2016 and it has always been referred to as CMPL-mutated ET, sometimes the C is lowercase., I have also noticed that this mutation is rarely mentioned.

I have not researched this mutation to any extent as I prefer not to worry myself too much about the technical aspects of this condition and just get on with my life.

Here is something I have just found from American Society of Hematology, November 2011:-

''Two cMPL mutations, cMPL W515L and cMPL W515K, have been identified in MPNs. The cMPL W515L mutation is reported to be present in ET and PMF patients, while cMPL W515K in some PMF patients.''

I don't know what the implications are, but I now feel prompted find out, maybe time to get my head out of the sand.

I am also 67, in the UK and on 8 grams of hydroxycarbamide per week.

[Quercus53 in reply to musicworks]

ooh dear sorry if I have prompted you to do research you don't really want to do. I always thought I had the CALR mutation but I discovered by looking at my blood test form that it cMPL. If you find out anything interesting, I would love to know as all the technical papers that have been written and published are like trying to read Vulcan!!

let's hope we get out of lockdown soon and get back to as normal as we are able

xx