I do not very often come across papers aimed at younger MPN patients so thought I would highlight this one that is being presented at the ASH conference in December. Nice to see that younger patients are being considered.
3033 Myeloproliferative Neoplasms in Young Patients: The Mayo Clinic Experience with 361 Cases Age 40 Years or Younger
"Most available data, including current prognostic models, are excerpted from older populations and may not accurately mirror the unique phenotypic and prognostic patterns seen in younger patients."
"When appraised as a function of MPN subtype, survival data confirmed the significantly reduced OS in PMF relative to PV and ET (P<0.001), though PMF patients aged ≤ 40 years were still projected to have a reasonably favorable median life expectancy of 20 years "
"Young MPN patients comprise a unique disease subset defined by an attenuated-risk cytogenetic and mutational backdrop compared to their older counterparts. Caution must be exercised in counseling and managing this population as historical ‘one-size-fits all’ data fails to reflect the true natural history and disease biology in the young."
Great news though a word of caution from an oldie... It is still important that younger patients keep up their visits with their haematologists, know their particular circumstances/risks and look after themselves, just like us oldies.
Good reading and best wishes ...Simon
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Thanks so much for posting this Simon. I’m 38yo, was diagnosed a year ago, and it is very welcome to see something on younger patients. Although I was familiar with MPNs and some of the treatments because of my Mother’s PV and secondary MF, being 20 years younger at diagnosis than she was, I feel a bit like a guinea pig. There seems to be no treatment for younger low-risk patients bar aspirin, so we have to manage the symptom load ourselves. I’ll follow this with interest. Thank you!
I’m on a bit of a rampage of late over the managing our symptom load ourselves bit! I don’t think this is acceptable. Many of us have a significant symptom load and we should be getting help with it. My hematologist blames my symptoms on being a mother of young children and newly forty. It makes me furious. I know what I am experiencing is not normal and no one will listen to me. I am trying hard to do everything I can for myself with exercise, over the counter pain medicine, supplements, etc. but my quality of life is not great.
Chelsea, I completely sympathise. After 4 years of battling with dismissive GPs for a diagnosis, I feel like I’m still shouting into the wind about my symptoms and quality of life. I know it’s partly because so little is still known and understood about these illnesses. I just hope someone finds a treatment for us soon. Meanwhile I’m going to try the CBD oil and keep my fingers crossed. If you need to offload, I’m here.
Thanks. I am interested to hear how the CBD works for you. I need to try CBD. I hear mixed things about whether it works without THC. Medical marijuana just passed where I live. It won’t be implemented for a couple years at least but I look forward to seeing more information come out about it.
thanks for this, very encouraging. As someone who was diagnosed with PV 30 yrs ago at 29yrs, I was initially given negative info re my prognosis but I'm still working full time!
Thanks for posting. I was diagnosed 2 years ago with pv at age 38. I have a 5 year old son and still panic about life expectancy sometimes. Does the comment about median life expectancy mean theyou think people might only live about 20 years after diagnosis? Sorry if I'm being a bit daft and thanks in advance. Tracy
ASH is a major annual conference for haematologists filled with leading information and research. When a haematologist discusses life expectancy with their patient, they will consider a patient's particular circumstances and how similar patients have fared in the past. The purpose of the paper referenced above is to help haematologists when they are having that conversation with younger patients. Essentially it is pointing out that younger patients have fared better than older patients so a different set of statistics should be considered when having a conversation about life expectancy with a younger patient.
Re the 20 year median life expectancy quote... that was for primary myelofibrosis (PMF). PV and ET have a better prognosis than PMF. I was quite surprised by the 20 year quote for PMF. Sounds much better than I would have ever guessed.
The 'median' only provides one bit of information. For example, the paper is saying that if 100 young patients were diagnosed with PMF, then in 20 years time one would expect 50 of them to still be alive. It does not indicate how much longer those 50 will continue to live. Nor does it take into account how advances in treatment will improve survival.
I think that the take away point is that you need to consider you own circumstances and that your life expectancy may be better than you initially thought. All the more reason to take good care of yourself.
Thank you. I'm a 33 yo with PMF. Using diet and exercise to regulate my protiens and so far, so good. I have no medical history before a spontaneous splenic rupture, so this was good to read. Appreciate the help!
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As a 26 year old who was diagnosed recently with PV I’ve been looking for a paper just like this!
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