Prof Claire Harrison shared ET paper via Twitter

[Magg]

sciencedirect.com/science/a...

[ksos]

Thanks! This is great -- Kim

[EPguy]

Thanks for the link.

This begs for more info "(recent data showed ET) prognosis has not improved in recent years"

Is there a link with more details of the "reference 3".

[Magg in reply to EPguy]

Hi EPguy

I’m wondering if this is 'reference 3'

I’ve gone down a rabbit hole with it as there are so many links 🤯 but can’t find further substantial information.

All the best Maggie

ashpublications.org/blood/a...

[EPguy in reply to Magg]

As with some other Dr Harrison info we've heard of recently, seems we will need to wait for publication of something.

[Xuzy in reply to EPguy]

I don't really understand the point of this. It's a given fact that no new drugs in recent years for mpn save ruxo and interferon have offered any deal breaking results and interferon which was already in use many many years prior to FDA approval, only recently showing data. And the overall course of the said disease is always studied based on those older. Now suddenly this data that yes unfortunately younger individuals are sure to die sooner is no new info. The prognosis has always been we don't really know who progresses and if they do how well can they take chemo or even respond to it is a data that is so individualised. The research or awarness on mpns is so slow that most doctors till date don't see it as cancers. Infact cml seems to have more treatment options than other mpns.

[Nmom]

My son was diagnosed at age 17 and all the drs said he should have a normal life expectancy. It was disheartening to read this article. I will not be passing it on to him.

[Trocken in reply to Nmom]

I’ve come away thinking exactly the same, I was diagnosed at 37, but going off my blood work they believe I’ve had since before I was 24 (not as young as your son) but it worrying none the less. I have always questioned how we can live a normal life expectancy when our body has had to cope with the disease for longer, it’s never made much sense, but this is the first time I’ve ever seen any data relating to life expectancy for those of us younger than 60 and if I’m honest is the information we have always wanted. It may not be something that should be kept from your son depending obviously on how old he is now. Xx

[Timjonze in reply to Nmom]

I would try not to worry. The life expectancy is much longer for those under 60 and I’d imagine within that group the figure given (32 years) would be longer for a 17 year old than a 59 year old. Even if it was 32 years that would hopefully take him to 49 …. A long way off and it’s impossible to say how many more treatments would arrive in that time

[azaelea in reply to Nmom]

I agree with you Nmom, it was disheartening to read this article as I have always understood ET sufferers should have a normal life expectancy.

[SilverET in reply to Nmom]

Hi Nmom,

Don't panic. ET seems to depend very much upon driver mutations as to how bad it is and how fast it progresses. I was diagnosed at 17 and am now 53. I have led a fairly normal life and do not suffer greatly from the fatigue etc that others describe, although my platelets at times have been over 1million. I know that I have some risks that others do not have but I also have the benefit of being prepared, knowing what my risks are, regular blood checks, some treatment options if needed and what symptoms to look for whereas most people do not know what they are likely to have problems with as they age.

A lot of the life expectancy data they quote depends very much upon the small group of people they study. There is always hope and there are always outliers that don't fit the numbers. When I was first diagnosed it was thought that young people didn't even get ET.

SilverET

[Nmom in reply to SilverET]

Thank you for the encouragement. My son is now 21. He works out and leads and active life. His diet isn’t great but I keep ‘planting seeds’ on healthy living. No mom wants their child to have extra adversity in life. I appreciate you taking time to post about your success. Any info on things to do/look out for would be appreciated.

Blessings!

[CLONDARA]

Thanks for the link Magg. Good to pass on for my family to read.

[Scarlett500]

How do we get access to the full paper, i can only see snippets

[Michali45]

I agree with the concerns expressed above. I was told at the time of diagnosis that having ET does not affect life expectancy. I am 77 so it doesn’t worry me too much anyway but does make me see my illness in a different light. I feel for those who are young but, of course, one expects progress in treatment options as time goes on. I have never met Professor Harrison though I am a patient of her team. I believe that, luckily, I am a boring case so there is no need to see her. But I will bring this up with whoever I see for my next appointment in two weeks time.

[Metronome]

First of all, the publication can be bought for $120 through Rightslink, for anyone that's interested. Nevertheless, I guess that the bottomline is, prognosis has not changed although there has been (?) some progress in understanding what happens.

My impression is that the more they search, the more they find that individual genetics have a lot to do with clinical presentation and prognosis. So there's not one ET, there's a core dysfunction and the way this interacts with each one's genetic profile and the way this changes over time. So they're back to square one.

Age in general seems to be the decisive risk factor because usually as we age we accumulate mutations, some of which can interact with the one triggering ET. How long that takes, nobody knows. The JAK2 mutation has also been found in infants, the majority of which will not develop an MPN as they grow up. Most people get diagnosed later in life but the process might have started decades ago. Can you really use "years from diagnosis" as a reliable starting point to make a prognosis? What about other co-morbidities?

Genetic screening (and subsecuent targeted therapy) is what is most probably suggested but that has no real meaning if:

1. You cannot be sure that you have identified the exact mechanism through which proliferation starts.

2. You cannot predict future mutations and their impact.

3. You cannot come with therapeutic agents until #1 is sorted out (at least) and you can have some model of predicting #2 to any degree possible.

The article would probably speak about the exciting new therapy trials which have been showing promising results on mice but are still far from being formed into medication and for sure sure there's a brighter future ahead...which is basically what was thought 10 years ago...still first-line treatments remain unchanged for years and humble little aspirin continues to be the backbone of treatment.

Sorry to sound pessimistic but although I have the utmost respect for scientists and researchers, a paper on how you failed to use 10yr of funding and how you look forward to another 10yr of funding is not what most of us would be hoping for.

[Xuzy in reply to Metronome]

Exactly this. I'm always so sad we didn't even have interferon as first line treatment till 2021 when it has been known to be used since decades.

[EPguy]

Looking at this again, this part is notable:

"heterogeneity of this disease (ET), ... is largely driven by driver mutation status,"

This is unusually explicit on the importance of knowing about the mutation. There might be some new details in the paid version of this article. But it supports the modern idea of getting a full MPN gene sequence.