This is the (new retrospective)study that says young MPNs have higher incidences of transformation than previously have been reported.
We identified 444 patients, with median follow up 9.7 years (0-47.8)."We identified 444 patients, with median follow up 9.7 years (0-47.8)."
"Disease transformation occurred in 48 patients (10.9%, 1.13 % pt/year), usually to myelofibrosis (7.5%) with splenomegaly as a novel risk factor for transformation in ET (p= 0.000) in logistical regression."
Does this mean that if someone has mpn for px.25 years has 25% chance of progression?? This seems like progression is inevitable. Also -given that median follow-up was 9.7 years- these 48 patients progressed from 0years to 47.8, or to 9.7 years?
I would like to know what is the cumulative risk of someone with ET to progress. And how much higher is than previous studies. 1.13% per year seams really really high cause that means that someone who has MPN for 25 years has 25% chance of progression! This sounds to me like progression is almost inevitable! Also this study included pre-PMF patients?
While certainly not medical doctrine, I have seen several references to the “1% per year” estimate provided by some MPN specialists. This refers to the general estimate that, each year, risk of progression is believed to increase by about 1%. This coincides somewhat loosely with the 20% at 20 years, 30% at 30 years estimate for ET transformation to MF. Again, these are estimates. Everyone’s case is different.
Hi Aneliv9, I asked Prof Harrison and she replied: it isn’t as simple as saying that if someone has ET for 25 years that they have a 25% chance of progression, and progression is not inevitable for all ET patients, and we advise that you discuss your concerns with your own haematologist.
As usual there is no easy answer. According to this report, splenomegaly and Jak2 type mutation are risks with ET. So a normal spleen and CALR or MPL would be lower risk by this thinking.
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This report seems to be for "MPN" patients. This would be ET,PV, MF, pre-MF ET etc. They do mention ET separately but it seems only in context of new findings unique to ET. 3 patients had SCT, this is strong evidence there were other than ET in there since SCT is rarely if ever done on regular ET.
They do note: <<splenomegaly as a novel risk factor for transformation in ET >> and have a discussion of ET risk scoring.
Another important item missing is what, if any, treatment these patients were getting. Young patients may get less therapy to address risks while older patients get more aggressive treatment. In recent years we know thrombosis and other basic MPN risks are greatly improved with current care standards. Did these young patients get this type of care? More often than elders they don't.
They note hyperviscosity symptoms are risk factors. This condition is usually addressed by controlling blood counts per standard modern care (HCT, WBC etc) Did these patients get the current standard of treatment?
In another thread today is a discussion that high PLT is likely not correlated to thrombotic events.
Thank you very much for your answer. It really confused me. But what worried me most was the risk of progression. I couldn't find an answer on what is the percentage someone under 25 to progress. 1%pt/year isn't it to much?
Furthermore, 10.9% of patients that progressed is the TOTAL percentage in these 47.8 years of the study or until the first 9.7 years?
The statistics can be a bit difficult to sort out. As you already heard 1%/year does not translate into 25% in 25 years. It is much more complex than that. The other issue is that no one can take these studies and use them to determine their own prognosis. This data is applied to groups of people, not to individuals.
As Mazcd suggests, the best thing to do is to discuss your prognosis with your own MPN Specialist. You can consider your own prognosis based on a number of factors known to be associated with risk of progression. Know that there is not a definitive answer to your question.
Suggest that you also discuss your concern in terms of your own treatment goals. If you want to prioritize reducing risk of progression then that will guide treatment decisions. It was not until recently that this even became a focus. We are learning more about how to reduce risk of progression thanks to the research underway.
There are some things you can do on your own that may help reduce progression risk. These are the same things that lead to a healthy lifestyle and reduce other health risks. There is no downside to doing any of these things.
1. Eat a healthy diet and avoid contaminants in the food stream.
2. Avoid carcinogens (environmental and ingested).
3. Control systemic inflammation.
4. Maintain healthy weight and fitness level.
Please do let us know what you learn when you consult with your MPN Specialist. All can benefit from what you learn.
<<From a strictly clinical point-of-view, we first confirmed that triple-negative ET is a very indolent disease, with a low incidence of vascular events as only two patients suffered from thrombosis. Furthermore, no case of fibrotic progression or leukemic evolution was registered, thus confirming the different behavior of this condition when compared with triple-negative PMF.>
If you want further details on your individual case, you can ask Dr for NGS gene analysis to look for non-driver mutations.
I remember quite a few of your posts and I hope I'm not overstepping the mark by observing that your main issue is anxiety that you might at some point progress to Myelofibrosis. I don't believe that analysing studies and statistics is going to help you. Try to live healthily, discuss the best treatment with your MPN specialist and most importantly, please try to get help for your anxiety.Imagine reaching the age of 85, having ruined your life by worrying constantly about your health. Think of all the joy in life you are missing.
When my sister had breast cancer, she saw a psychologist who told her that she had seen far more lives ruined by fear than by cancer.
The fact is that most patients don't progress to MF. If you eventually do, treatments will be even better than they are now.
Please, please get some help for this. I'm not unsympathetic - two people close to me have suffered with severe anxiety.
We also experience fear at times. I have had a MPN for over 30 years, with the last several years offering various medical adventures including progressing from ETto PV, heart and brain surgeries, and more. There are a number of things I have found that help.
Say the Serenity Prayer every day and implement it. Change what you can and let go of the rest.
I sometimes recite this quote and take it to heart.
"I must not fear. Fear is the mind-killer. Fear is the little-death that brings total obliteration. I will face my fear. I will permit it to pass over me and through me. And when it has gone past, I will turn the inner eye to see its path. Where the fear has gone, there will be nothing. Only I will remain." Frank Herbert, Dune
I have been down the road of letting the stress overwhelm. It is not a good place to be. Note that some seem to find that unmanaged stress increases thrombocytosis. Stress is a killer in more than one way. Stress also kills joy, something we all need in our lives.
Know that you have this. The probability is that he ET will not progress into MF/AML. there are things you can do to reduce your risk. Equally importantly, you can live a great life while managing ET. Despite 30 years with ET/PV and various other issues, I have lead a great life and continue to do so. Please plan to do the same.
Thank you.. It is like a gambling game Living with ET. I don't really know what are the chances of progression in let's say: 50-60 years of living with ET and i can't understand some statistics of studies. Do you have an idea??
The answer gets into the specifics of research methodology. in order to achieve a statistically significant result there needs to be a large enough sample size. In addition, the sample needs to be homogenous. All cases of ET are not the same. Some have non-driver mutations. Some have have a different driver mutations, Some start at a different variant allele frequency. Some have co-occurring medical conditions. Many have different lifestyles that may promote/reduce risk of progression. In addition, some current studies include people misdiagnosed as ET who were actually PV or prefibrotic MF.
In order to get a valid statistical prediction of your individual risk based on research you would need to be able to compare to people diagnosed near your age , with a similar genetic and medical profile, and who follow the same treatment plan, living the same lifestyle. In addition, the study would have to start now as opposed to having started 20 - 50 years ago. It is not a valid assumption to propose that someone initially diagnosed 30 years ago would have the same outcome as someone diagnosed today. There are too many variables, including the improved medical care we have now.
In addition, these studies are simply not designed to follow people that far out. Longevity studies longer than 5-10 years have to be done retrospectively, with the intrinsic limitations of retrospective research. Therefore, you are not going to find a definitive answer in the existing research.
What you can do is make a generic theoretical projection of your long-term risk of progression based on assumptions that may not be valid. It is nothing but a guess. Just for the sake of argument let's assume the long-term risk at 50 years of progressing to MF/AML is 20%. Think that through on a couple of points. That means that there is an 80% that you would not progress. In addition, consider life expectancy averages (roughly 81 female/76 male). If you are diagnosed in your 30s, statistically you would likely be at end of life 50 years out from point of diagnosis regardless of the ET progression. The bottom line is that you are more likely to die with ET than from it.
As you may recall, I have been living with ET for 30 years and it did progress - into PV 8 years ago. I also have a non-driver mutation (NF1). The NF1 makes progression to AML more likely. The NF1 caused a brain tumor requiring surgery I also have an arrhythmia that required heart surgery. It may still progress into A-Fib. I have a number of other medical issue too.
What I have found is that there is no point to worrying about what may happen. Knowing whether the risk of progression is 5-10-20% does not change anything. The intrinsic risk is not something we can change. What we can do is change the things we can to achieve two basic goals. 1. Reduce risk of progression (live longer). 2. Live a good life (high quality of life). Of the two goals, I find that the second is the most important. it also tends to support the first goal.
I would suggest finding a way to refocus your concern on improving your chances and living a good life. You are going to live a long time. Live well and embrace life. Part of how I do this is to start every day with the Serenity Prayer and embrace what it means.
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Simple Tree for MPN Classification Via the 'Machine-Learning Model' – ML
