MPN-U

[Paul123456]

Got my BMB results and dx is PV or MPN-U. Not come across MPN-U before?

Does anyone else here have this condition? Wondering what the treatment plan is, I read somewhere that prognosis poorer since trickier to treat

[lizzziep]

Not heard of MPN - U before - it could be that they know you have an MPN but aren't quite sure which one it is. Hope you can get some positive answers.

Best wishes

Lizzie

[Bluetop]

I believe I have read of someone else with MPN-U on this forum, some months ago. I'm PV but have not had a BMB, so it sounds like the MPN-U might be something that can only be identified through that. Hope you get more info soon.

[mhos61]

Does the U stand for unclassified?

I think there are people on the forum with PV who have not shown positive for any of the known mutations.

Mary x

[Indigo42916]

I have only heard of MDS-U?

[katiewalsh]

Hi. I think at one clinic I’m classified as MPN Unspecified. Meaning I don’t clearly fall into just one type. I could be ET, pre-fibrotic stage of MF & one other thing. But generally they consider me ET but my specialists both say the classification isn’t important at this stage because it doesn’t affect my treatment. Not sure if this helps you. Good Luck. Katie

[Paul123456 in reply to katiewalsh]

Thanks for all the replies. I saw one hem yesterday who wasn’t sure. Said looked like ET slowly changing into PV but when I said what’s the difference between U and Masked PV, he said ‘Semantics’.

Like you, I’m obviously concerned that U could also include ET progressing into MF. I’m my case, my Allele Burden (% JAK2 mutation) is 60% whereas PV averages c. 45% and ET 25%. This predisposes me to progression although it is not a given, just loads the dice a bit.

Hence my great dilemma at the moment, do I start Pegasys which can lower AB according to its supported. The amazing thing is that half the world’s renowned experts (incl Silver, Hasselbalch, Kiladjian) say yes, must start Pegasys asap whereas the rest (Tefferi, Vannuchi, Barbui) say doesn’t slow disease progression and effectively HU first choice, Pegasys second. They produced a paper this month based on available clinical data that supported this, incl their claim that HU does not increase Leukmatic progression risk.

It appears that when/if we get MF, the form will depend on our chromosomes. If we have 1q, 12q, MDM2 and MDM4, then this binds up p53 which causes more DNA damage and fast tracks progression. Hence this may explain why some people can live with MF for many years. As someone more at risk, I’m investigating how to get this test but early feedback is hideously expensive!

Incidentally, this new drug, RG7388 targets the above. Very early stage trials but promising.

[zvinkovic in reply to Paul123456]

Hi Paul,

HU is first choice because it is cheaper and much easier to take. I would be interested to know are there studies comparing overall survival, complications and progression of disease depending on treatment.

I agree that maybe HU doesn't increase the risk of leukemia, because it is used as treatment for children with sickle cell disease, but the question is how it affects overall survival. There were studies that showed that interferon lowers JAK alele burden, and also to reverse existing myelofibrosis and for me there is no question between HU and INF, INF is superior in every way. Also since I was put on INF other counts are normal, while when I was on HU my hemoglobin and red and white blood cells were low, so I would pick up every virus there was and would feel dizzy and pass out frequently. If you are offered Pegasys, take it, you can always switch to HU if it doesn't work out for you.

(I was diagnosed with ET in 1999, on interferon since 2004)

[katiewalsh]

Have you looked at Patient Power for results of 2017 ASH conference? It’s the leading hematologists in the world, reporting on latest news. You know much more than I do. Good Luck. Katie

[Paul123456]

Yes, I follow PP, it’s very good.

Re knowing more! I think the more I find out I realise the less I know!

[Garden987]

Hi Paul. Sorry I can't be helpful re your query but I want to ask for your help please. I think it was about 9 months ago when you gave the name of a website called targetdonc.com/conference/ where Prof. Claire Harrison was talking about MPNers still having symptoms even though their bloods were OK. Have tried the website but can't find Prof. Harrison's talk. Could you perhaps guide me there as I want to show it to my haemo. Many thanks. Mary

[friendofpiglet in reply to Garden987]

This might be it.

targetedonc.com/conference/...

[Garden987 in reply to friendofpiglet]

Hi. - interesting name by the way! - thank you so. Much for your reply. Couldn't link into previous blog even though I spent ages looking. Found it very comforting in a sort of way because although my bloods are "perfect" - platelets now 230 my haemo appears not prepared to accept what PRof. Harrison is saying. NEeded this info. to show her and others. What a complex condition we have. Again, thank you very much and I send you my very best wishes. Mary

g

[Paul123456]

Sorry Mary but wasn’t me. I’ve only been posting a couple of months Best Paul

[Garden987 in reply to Paul123456]

Thanks for taking the trouble to reply Paul All good wishes. Mary

[Mazcd Partner MPNVoice]

Hi Paul, I asked Prof Harrison for clarification: MPN U means not enough features of one specific disease, often will evolve to one of the entities – ET, PV and MF. Does not necessarily imply more difficult to treat or worse prognosis (though there is some literature on that). best wishes, Maz

[Paul123456 in reply to Mazcd]

Thank you Maz for taking the time to ask, very kind

Best wishes Paul