What is the future treatment for polycythemia vera

[Spa1981]

I just got confirmation that I m jack2 positive. Bone marrow findings are suggestive of panmyelosis and my rbc, hct, hgb, all were high. I m 40 yrs old and hv also chronic liver disease due to hepatitis b. With the help of new generation antiviral I was just controlling that disease at that time I got this news. Does it hv impact on liver. What is its life expectancy? I did my first phlebotomy and rt now hct is 40, and platelete 430, do I need to take aspirin? I hv erosion in stomach so thinking twice before taking it. is there any promising therapy under trial at present for this disease?

Thanks

[hunter5582]

Sounds like you are on the first steps of what will be a long MPN journey. The good news is that for most of us it is a long journey, albeit with some challenges along the way. There is a lot to learn to effectively manage PV. Here Voices of MPN website is a really good place to start. Here is a link to some presentations what will also help you start to learn about MPNs and PV treatment.

mpninfo.org/conferences/201...

Many people with PV do benefit from a blood thinner to prevent thrombosis. If you have stomach issues there are alternatives to aspirin. This is something you need to talk to your hematology provider about. NOTE: be sure to consult with a MPN-expert doc. Most hematologists lack the KSAs to provide optimal treatment as MPNs are so rare. Here is a list mpnforum.com/list-hem./ .

A few quick factoids. JAK2 = Janus Kinase 2. It is a reference to the JAK2v617f (or exon 12) mutation in hemopoietic stem cells (HSC). The JAK2 mutation is a gain-of-function mutation that causes HSCs to have the "on switch" always on. This causes the overproduction of red blood cells (and sometimes platelets and white blood cells). It can also cause the overproduction of inflammatory cytokines that are responsible for many of the secondary symptoms we experience. (including stomach/inflammation)

Yes PV can effect the liver, but more commonly the spleen. Talk to your providers about this. Some of the meds used to treat PV can be hepatotoxic if you need them. Be sure to get your providers to collaborate on this too.

Life expectancy - most people with PV live a near-normal life span. It depends on a variety of factors and there are different risk bands based on your individual risk factors. At age 40, you should have a long time to sort this all out. There are promising treatments in development that will likely benefit you in your lifetime.

FYI - I am age 65 with JAK2+PV. Was diagnosed with ET about 30 years ago. It progressed to PV about 7 years ago. Still alive and kicking. Despite a few challenges of late, I have had and continue to have a good life. Don't buy into all the gloom and doom stuff you may encounter regarding PV. There are challenges to managing PV, but believe you can successfully manage it and have a good life.

All the best to you.

[Spa1981 in reply to hunter5582]

Thanks for ur insightful reply. In the comment section of my bmb test written that suggestive of panmyelosis but in investigation I found myeloid to erthryoid ratio is increased. Is it possible in pv? Or do i need to perform more tests to find the reason like bct abl test. Is it possible to have both disease at the same time I just wonder.

[hunter5582 in reply to Spa1981]

I really do not know enough about your situation to even hazard a guess. Have you looked into MPN/MDS Overlap Syndrome? There are also times when ET or PV is progressing into MF, but I have no idea is that applies to your situation. I would be really sure to consult with a MPN Specialist. A regular hematologist is really no likely to meet your needs.

I hope your get some answers soon. Please let us know what you find out. Shared knowledge makes us all better equipped to deal with out MPNs.