We are all different in how our MPN presents, including in the potential for progression. There are a number of factors that influence the presentation and progression of an MPN. These factors include the specific mutation that causes the MPN (JAK2, CALR, MPL, triple-negative), the Variant Allele Frequency of the mutation, whether the mutation is heterozygous or homozygous, presence of non-driver mutations (e.g., ASXL1, IDH1/2, TP53, and more). and co-occurring medical conditions. There are likely other factors that influence progression like lifestyle choices and environmental exposures.

So the answer to your question is that all 10 people with ET could present differently in term of their symptoms and rate of progression. This speaks to why it is so important to receive individualized MPN care by a MPN Specialist.

So when I got my diagnosis of ET Jak2, I googled it and it said the average lifespan for someone with ET is about 20 years. There are many who’ve had it longer. I think they were referring to the fact that most people get this late in life. I was 52 when I was diagnosed in 2008. In 2019 I was diagnosed with post ETMF. I was told at the time that I would need a stem cell transplant within 3 to 5 years. So I feel that for me, the 20 years approximation is true. Some people never morph and for those people, they will probably live a lot longer. Everyone is different.

Let's say it is far more likely that the 10 people will end up with different trajectories of illness than they are to have similar trajectories. Age at baseline matters too, and so does biological sex, it seems.
https://www.sciencedirect.com/science/article/a...

As Steve already mentioned, the driver mutation behind the MPN can influence illness progression, but this in itself does not explain the large variation in illness outcomes in MPNs.
This because there are many other genes that can harbour mutations (non-driver mutations) that can increase the risk for transformation. Some of these genes we know about. And we are learning more and more about other genes. People can also harbour other mutations that can predispose them for solid cancers that can also increase the likelihood for an MPN.

A Danish study showed that ~3% of the studied population had a JAK2 pathogenic mutation, yet only ~3% of the people who had this mutation had MPNs.
https://ashpublications.org/blood/article/134/5...

As Steve already mentioned, some environmental and behavioural factors can also increase the risk of developing the disease, but by themselves might not fully explain progression. Smoking increases the risk for developing MPNs, so does working in a high-risk environment for long time. Having chronic inflammation due to another illness, for example autoimmunity, increases the risk for developing MPNs, and in some people even the risk of progression. But even there, there are other factors at play, such as other pathogenic mutations that might not be directly linked to MPNs, but they can influence outcomes. Treatment for another cancer might also increase the risk of transformation.

There is also the fact that, for some people, blood cancers, both acute and chronic, run in the family, although they are not inherited. What one inherits is the propensity to acquire a pathogenic mutation during lifetime. The genes responsible for that propensity vary, which means that the illness outcomes when a pathogenic mutation is acquired might also vary. Even within the same family there is significant variation in disease trajectories despite having the same driver mutation.

I know it is frustrating not being able to predict how this illness will go, that there is so much uncertainty. One can only hope we will have more answers sooner rather than later.

Hi, I think we all differ, some progress quicker then others, and some can go into remission, I personally know of a person in remission, its a wait and see Antoinette best of luck

in 1995 the survival rate for pv patients was 7 years