Mpl mutation

[Cookiebaker]

I got results from genetic testing for ET and my mutation is mpl . I know this is the rarest of the three mutations and it is the one that most often morphs to myelofibrosis . What else can anyone else tell me about it? I want to find out as much as I can . My sister passed from MF 9 years ago before they knew of this mutation so I don’t know if she had it too. Thank you.

[mjfin72]

Hi Cookiebaker. I have ET diagnosed in 2016 and also have the mpl gene however I did not know that having this gene could be more likely to transition to MF. All I was told was that it was the rarer of the three mutations but treatment plan would be the same.

I have been on Hydroxy since last Feb and responding well to treatment bar a few blips along the way. Would be interested to know more about the likelihood of transition with our gene mutation.

So sorry to hear about your sister. I'm sure having someone close pass away with MF causes you to worry more.

Regards

Mandy

[Cookiebaker in reply to mjfin72]

I do worry about the future after being with my sister so much during her illness and death. Thank you for writing.

[Paul123456]

I’ve not seen any research to indicate that MPL has an inferior prognosis to JAK2. Last research I saw stated similar life expectancy. Hence perhaps you can clarify with your Hem on your next visit. I can send you link to research.

CALR Type 1 has best prognosis but perversely, does increase risk of progression to MF. However very slow progression thereafter.

Best Paul

[Cookiebaker in reply to Paul123456]

My doctor gave me a copy of study that was published in Blood Cancer Journal . There were 665 ET patients in the study. The transformation rate to MF was 33% with driver mutation of MPL compared to 11% in triple negative, 15%Jak2and 21% CALR.

It doesn’t say anything about life expectancy in this article.

Thank you for writing. I enjoy reading about what others know about this disease.

[Paul123456 in reply to Cookiebaker]

This is the link I was referring to

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

Best

Paul

[Paul123456 in reply to Paul123456]

See page 11

‘Although the prognostic models for PMF currently used in clinical practice are essentially based on clinical and hematologic parameters, recent observations indicate that the driver mutation has an independent effect on overall survival and risk of leukemic transformation.16,84,85 In our study of 617 PMF patients, median overall survival was 17.7 years in CALR-mutated, 9.2 years in JAK2-mutated, 9.1 years in MPL-mutated, and 3.2 years in triple-negative patients (Figure 4A). Notably, triple-negative patients had much higher incidence of leukemic transformation compared with either CALR-mutated or JAK2-mutated patients.’

Remember this data is very historic, new treatments will enhance prognosis. Plus a median including older patients with higher rates of progression

[Cookiebaker in reply to Paul123456]

Very interesting Paul. Thank you so much for the link and sharing.

[Tico in reply to Paul123456]

Hi paul,i agree a lot of the data is historical and new treatments will enhance the prognosis along with new discoveries and more knowledge.thank you. Atb, tina.🤗

[Aime]

Hi, I think it’s also important to remember we are all individuals with probably unique medical histories so just because one person has not survived as long, does not mean another will be the same.

Sorry to hear about your sister, sending E hugs to you. Kindest regards Aime xx😺

[Wyebird]

Hi Cookie,

Your head must still be whirling with information and emotional termoil. I wish you well. Reading your post has enlightened my knowledge.

1- I’ve never heard of MPL mutated gene and I’ve been to lots of MPN forums. I’m CalrET.

2- I thought that Calr had the best prognosis but after reading the posts below it seems it’s the one most likely to transform if you are on hu.

3- if you’ve the MPL gene what do you have, ET, PV or MF?

[Cookiebaker in reply to Wyebird]

Hi Wyebird, I have ET. Hope that it stays ET for a long time. I am 70 and have had high platelets for 4 years. I had just been on baby aspirin until 6weeks ago when platelets reached nearly a million. In the 6weeks of Hydroxyuea they have dropped to 636 with a dose of 500 a day. So good results. I am trying to stay positive... but find myself thinking of my sister and her MF.

[Wyebird in reply to Cookiebaker]

Meds for MF seem to be developing all the time. Watching your sister suffer must have been a double wammy. I wish you well.

[Cookiebaker in reply to Wyebird]

Thank you Wyebird .

[Indigo42916]

Just saw this, and I am ET and MPL, and I understand the chance of progression to myelofibrosis about 30%. I am doing very well on Pegasys, platelets down from high 600's to 200's and feeling great. Good studies on Pegasys and normalizing bone marrow. My rbc's remain high normal and being watched.

Take good care.

[Cookiebaker in reply to Indigo42916]

Hi Indigo, I have a 4month check up next week. My rbc wbc are both below normal. I take Hydroxyurea. I will watch for your posts since you have the same mutation as I do. Thank you for writing.

Cookiebaker

[Indigo42916]

Likewise, thanks for sharing, the MPL's are a small lot, not much data for us.

[Indigo42916]

Hi Cookiebaker,

Just wanted to check in and see how you are doing.

Hope all is well.

[Cookiebaker in reply to Indigo42916]

I am doing fine . How about you?

[Indigo42916 in reply to Cookiebaker]

Sorry for the very late reply, doing well had the flu, but got through it. Energy is slowly coming back. Hope all is well with you.