Allele burden 26%

[Sprat19]

MPL Et just seen BM result which says MPL allele burden 26% not quite sure if this is high or low or not important been offered HU or peg interferon trying to research. Thanks

[hunter5582]

MPL is the most rare of the three MPN driver mutations. With JAK2, 26% would be considered in the low-moderate range. Anything under 50% is associated with a lower disease burden; however, this is just a tendency. We are all different.

At age 61 you are on the borderline for being considered high-risk based on age alone. Some docs still use age>60. Others have switched to age>65. Other pay more attention to your individual profile than age alone. My MPN Specialist told me "65 is the new 35." I like that doc! you will have to decide for yourself whether the benefits of cytoreduction outweigh the risks in your own situation.

Regarding your choice for cytoreduction, HU and PEG are the two first line choices. Each has its own risk/benefit profile. We each respond differently to each of these choices. I am very glad to hear you have been offered both. Once you have done your research you can make a decision based on your goals, risk tolerance, and individual preferences. Here are a couple of articles that may help.

mpnjournal.org/how-i-treat-...

legeforeningen.no/contentas...

ncbi.nlm.nih.gov/pmc/articl...

FYI - I was diagnosed with ET about 30 years ago. It progressed to PV about 8 years ago. I have tried HU in the past, but could not tolerate it. I experienced toxicity even at very low doses. I am currently taking Pegasys 45mcg/week. It is controlling the thrombocytosis and erythrocytosis. I have not experienced any adverse effects after 5 months on the PEG. Do note that is my experience. Not everyone tolerates PEG as well as i have. We are all different in how our MPNs present and how we respond to the meds.

Good luck on this journey. Do please let us know what you learn and how things go.

[Sprat19 in reply to hunter5582]

Thanks Hunter .That is really helpful

[EmeraldA]

Hi! I have ET Jak2 positive and my Allele burden at diagnosis via a blood test was 20%. I'm 46 at present and have not started any treatment other than aspirin. I will add that although I got my AB % I am not really sure what to do with it at the moment other than use it as a baseline and know that it isn't too bad a percentage to have. Bx

[JT_Marlin]

I’m 41, Jak2+, PV…I had a heart attack at 36 and stroke at 40 - both presumably from clots tied to my PV. My allele burden at 40 was 6% and 10% at 41. Will get it checked again in Jan.I started on PEG 45mcg/week at 40 for about a year and a dew months ago bumped up to 90mcg/week. When starting it originally and then again when bumping up the dose I definitely went through an adjustment period of a month or two of extreme fatigue - otherwise not too bad.

Liver enzymes tend to be outside normal range so need to limit the alcohol in take somewhat.

[EPguy]

As Hunter said, MPL mutation is less common than ET mutations Jak2, CALR. So there is less knowledge about it. According to this reference:

nature.com/articles/s41408-...

<<MPL-mutated ET is uncommon, with a variable but typically cited incidence of less than 5% >>

<<Further, MPL-mutated ET cohorts have higher reported rates of fibrotic progression than their MPL wild-type counterparts>>

In the case of Jak2 mutation PEG INF is considered a first line option in Europe. In the US it is less prescribed but for all of us, the new Ropeg interferon may in time be a first choice. HU has minimal effect on allele while it can keep your blood numbers good. (I have great blood #'s but still want to get off HU for long term benefit and quality of life)

INF can often reduce the burden % with JAk2 but I can't find info on MPL. If it is similar for MPL your Dr may recommend it as a way to reduce your risk.

From what I've seen on this forum, Dr Claire Harrison in UK is a top expert to help with your decision. She is noted in the reference Hunter sent you. In the US, Dr. Richard Silver's group in the same reference is a pioneer in INF (such as PEG) use and his institution has extensive INF info.

Considering the info in the reference on fibrotic risk here I would suggest you contact the top experts to discuss INF and other options.

In the table pictured here from

mpn-hub.com/medical-informa...

INF has a strong effect reducing allele compared to HU, which has no long term allele benefit. But this was not studied against MPL to my knowledge.

Ropeg Allele

[Sprat19 in reply to EPguy]

Thanks. I will have a good look. I have been struggling to find many statistics with regard to MPL ET. I know it is more prone to progression on paper but as the rare form of the rare disease it seems hard to find statistical significance. The progressin is one factor that’s making me lean towards peg

[Bmj63 in reply to Sprat19]

Hi i was diagnosed with Mpl et in 2020 and like you have struggled to find information except for the high risk of progression to mf. I am on hydroxycarbamide 1000mg day.was not offered INF. I am 76 yrs old with heart disease and copd.

[Aneliv9]

With mpl mutation, could i ask you what are your blood count numbers? Let's say do you have high hematocrit or hemoglobin? In what range are your.platelets?