Something for everyone - MF-PV-ET

[hunter5582]

From our friends at MPN Advocacy and Education International - Recordings from recent Conversations with MPN Specialists

MF

youtube.com/watch?v=vaGhCPf...(Not working as expected?)

PV

youtube.com/watch?v=u0jVLdh...(Not working as expected?)

ET

youtube.com/watch?v=OqdDQug...(Not working as expected?)

[kamiilos]

Thank you for sharing, it's always good to educate myself further.

[Anouchka]

thanks Hunter. The PV one was very informative!

[Tintins]

Thank you. A really helpful discussion.

[gardner99]

Many thanks for sharing the informative videos. It's always good to be reassured that having ET isn't the end of hope for a "healthy" retirement.

[champ30]

Thank you Hunter... found the discussion on MF really interesting. Enjoy your Easter holidays .Lynn

[Spanelmad]

Bought up lots of questions for my Guys appointment, so thankyou

[lakeview65]

Hunter, Thank you for posting this informative podcast. I listened to it on my walk today. It reminded me that we need to take charge and make sure we are also eating right and exercising . Never not once has any of my 2 hematologists or my MPN specialists ever said a word to me about my general overall health and how we benefit from healthy choices. The thing is, I don't have any symptoms and my CBC is all good for now so they tell me I am easy or boring.. for now anyway. Happy Easter all!

[hunter5582 in reply to lakeview65]

Healthy choices are all the more important when we have a MPN. There are huge benefits from making healthy choices. Glad to hear you are being boring. That is my goal. I want to be "The World's Most Boring Patient."

[EPguy]

Here are some notes on my take for the PV talk. Good discussion of some points in recent threads.

--

Notables:

IFN and now Rux are considered disease modifying

Risk factors in PV, familiar factors including Jak2 level. He specifies high and esp increasing WBC levels.

Rux is by far the best option for itching

Clarified the reported limited time on therapy for Rux (ie 3 years) is for MF, PV does not share this limit.

None of the newer Jaki-s are compelling for PV.

--

--

Misc notes: (with a few comments)

Till few years ago Tx was only preventing blood clots. (Plb)

Now we can impact the disease via IFN (Bes).

Goal is to eliminate Plb.

Hepciden Memetics (rusfertide, there is at least one other) Give better HCT control. These "lack disease modification"

Rux now we are learning (Majic PV) that Rux like IFN it reduces Jak2, and these pts get best EFS.

Both Bes and Rux are improving EFS progression to MF and AML

--

--

Questions:

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Combos:

While MF is using combos, PV it is not established, except Hepcidin drugs (eg Rusfertide) are specifically good for combos

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Brain Fog:

It's part of the MPN, less than from the drugs. There are no drugs to help this. HCT control is the only suggestion.

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Itching:

Rux is the best for PV itching, his experience supports it. One pt requires 20BID for itch relief but this high dose is exception.

Anitihistamines etc for itch but Rux is best

--

Other Jaki's not exciting in PV:

Momelot: not promising in a PV study

Fedrat, Pacrit no serious studies, weak signal in PV

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Questioner: Rux pts go off 50% within 3 years via worsening or progression AML.

Dr says:That statement applies to MF. PV does not fit this description.

PV direct to AML is possible, likely with hi risk non drivers.

IFNs maybe good for hi risk, but IFN does not work as well with hi risk mutations.

Hi risk can be specific mutations or hi WBC.

IFN guidance benefits from long IFN history

He minimally notes PEG in its use for PV, largely focused on Bes for the IFN.

--

Younger ET, PV pts have best survival

IFN makes a big diff in young.

--

VAF vs risk "It does correlate. Higher progression, 50%+ VAF venus thrombosis. Is reducing it helpful? Most Drs don't check regularly. Need more data. Kiladjian says if you reduce to less than 10% and CHR you can stop IFN (My single experience does not support this)

--

VAF can be read from blood and marrow, equally reliable (but my experience the value can be very different)

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Neuropathy: Its not common but he does hear about it. But it's not likely from IFN, Rux or HU.

Bone pain is well known MPN Sx.

He rec 2x aspirin/day for those with micro vascular issues, current MDA policy.

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HCT by gender: MDAnderson does not use a different value for female all are <45.

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PV correlates to arterial disease s well as venous

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Fatigue: most common MPN Sx. It's pervasive across all cancers. (and autoimmunes) No treatment addresses it.

Weight gain is from Rux, usually minor.

Some PV pts report prolonged illness recoveries, Dr guesses Rux might be a factor but treatment histories are not known here.

--

~1% PV pts are not Jak2+

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PEG+ Rusf: (one explicit mention, from questioner, of PEG) IFN is slow, so Rusf could be great adjunct for immed HCT control. (or for HCT non responders)

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Should PV with no iron stores take iron supps-usually not. Exception may be those with phlbs.

--

MPN might add to kidney stones, a theoretical answer only.

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High Jak2VAF and 2ndary mutations: how to prevent progression . Increasing WBC is a signif risk along with hi VAF. Only two drugs can improve EFS- Bes, and Rux.

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Is a cure possible?: H-Jaki's (this very interesting concept is muddied and not explained) LSD-i (Bomemdstat)

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SCT is not a PV option, too risky. MF gets SCT if less than 5 year outlook. (some Drs suggest getting SCT before MF things get too bad)

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Does he see many pts on HU with mouth sores: "not alot" More common is too low counts and he suggests Rux ( an HU dose reduction would make sense, he does not explain)

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Life expectant with PV: median in two large studies 18-19 years. Age, white count, thrombosis,. Requires individual details to be useful.

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Is BMB required: for Dx yes. To Dx ET vs PV, prognosis if fibrotic, after Dx if there are risky changes/. BMB also can be to rule out PV, ie 2ndary PV.

--

IFN and Rux are disease modifying, HU is not.

[Manouche in reply to EPguy]

« Kiladjian says if you reduce to less than 10% and CHR you can stop IFN (My single experience does not support this)« 

He said that patients who manage to get a complete haematological response for at least 2 years, no phlebotomy, and a jak2 AB < 10%, can stop interferon treatment for a few years.

[EPguy in reply to Manouche]

Thanks for the details/qualifiers, this was a 2ndhand quote in this talk. It didn't recall the stop had a limited time, I think a Dr Silver talk somewhere proposed very long term cessation but I recall presented no data. My guess is these are the lowest VAFs ie <2% or so which we've seen used as a cut off for some reports.

I met the 2 years CHR but only with the year on HU included. I'm sure they mean all via IFN. I got three months of maintained CHR post IFN.

[Innessant]

Thanks for the editing and sharing this. Much appreciated.

[PhysAssist]

Thank Hunter!