PV progression?

[Steve_Essex]

Hi,

I’m 39 years old and was originally diagnosed about 4 yrs ago with ET.. platelets around 550.. steadily increasing to 850-900s and recently (last year) just over the 1000s. I had a BMB done with no fibrosis detected.. also gene profiling confirmed only JAK2 with no other mutations etc. Allele burden was around 17%

I’m under the care of Guys and they noticed my hct Levels were borderline, a red cell mass test confirmed 33%, which is over the recommend levels of (I think) 25%… Hct is at 45% so just on the border to recommend venesections.. which I’m having every 3 months at the mo..

I’ve not had an official PV diagnosis, partly as when discussed my BMB had ET characteristics but being treated ‘as PV’ due to HCT and red cell mass…

Recently started developing pruritus which is mild in mornings, and worsens with showering.. also my last 2 blood tests over last 6 months have shown a decline in platelets without cytoreductive therapy… 1050 -> 920 and then 920 -> 620… back Jan 2022 for another blood test to see where things are at…

Haem didn’t seem concerned and I initially left feeling quite happy with the latest results… however I’ve since read that falling blood counts can be associated with early MF progression, which I’m obviously concerned about… but would that generally impact all blood counts, not just platelets? I.e Hgb? WBC?

Apart from the itching, which I control well with Beta Alanine daily.. I’m feeling good in myself… train 4 times / wk… in good shape etc..

Guess I’m looking for a bit of perspective from the collective experience of the community!

Thanks in advance!

[hunter5582]

I would not interpret your reduction in thrombocytosis to be progression if you are on cytoreductive tx. That is the purpose of the treatment. Prior to being on my current treatment, my platelets would cycle by as much as 250K. Bear in mind that platelet levels can vary by as much as 100K in a single day based on what is going on in your body. this is normal.

I was diagnosed with ET about 30 years ago. It progressed to PV about 8 years ago. Progression can occur and can be gradual. There is also the condition known as Masked PV which I expect was the case for myself for longer than 8 years ago.

I went on hydroxyurea back in 2018, but after a year could not tolerate it. I experienced toxicity even at low doses. I did phlebotomy-only for nearly 2 years, but the chronic iron deficiency became problematic. I went on Pegasys back in May 2021. At 45mcg/week I have attained control of the erythrocytosis and thrombocytosis with no side effects at all.

One of the driving considerations in my choice for PEG is the solid evidence that it is the one drug that is potentially disease altering. In addition to excellent symptom control, it gives me the best chance to prevent progression of the PV.

If you are at Guys I would imaging you are being seen by a MPN Specialist. Your concerns are worth discussing at your next appointment as are the options you have to reduce the risk of progression.

All the best to you.

[Steve_Essex in reply to hunter5582]

Thanks Hunter,

I’m planning on speaking to the specialist if I can get hold of them today as should have raised when I got my last results three weeks ago… but initially took the drop as a good thing!

Just to confirm, I’m not on any other therapy other than 75mg aspirin and quarterly venesections hence my concern as 400k is a pretty big drop in 6 months..

Thanks!

[hunter5582 in reply to Steve_Essex]

I misread the original post. I can understand why you would be asking questions if you dropped from 1050 to 620. I still would not assume this is indicative of progression into MF since you are still in thrombocytosis. I used to typically run in the 500s - 700s unless something was going on in my body to cause reactive thrombocytosis. Then my platelets could spike as high as the high 900s. There could be as much variation as 400K.

The pruritis sounds like a significant concern. I have not experienced that symptom but do get eczema, which is also quite bothersome. I found treatment that works to prevent/resolve the eczema. Hope you can do the same for the pruritis. PEG may help with that, but ruxolitinib is reputed to be the most effective treatment option for this symptom. Unfortunately, it is quite expensive nd difficult to access in some health systems.

I hope you get answers to your questions and resolution of the symptoms soon. Please do let us know what you learn.

[onPoint]

You could get (if not recently done) a blood count to check for any signs of increased glucose levels. Pruritus can be a symptom of diabetes. I have ET and my pruritus has decreased significantly since I controlled my sugar/carbohydrate intake.

[Cja1956]

You might want to see if you can get another BMB, if you’re concerned about MF. I was diagnosed with ET jak 2 positive in 2008. My doctor put me on HU immediately even though my platelets were only about 600. In 2016, my blood counts started changing and my platelets started to increase but my red blood cells started to decrease. In 2017, I developed anemia. I suspected MF, but my doctor said that wasn’t the case. He did put me on Ruxo but my anemia got worse. So I found a specialist in 2019 and she did a BMB and confirmed my suspicions.

My case is different in that my platelets remain high. She actually took me off Ruxo at the time and put me on hu and Fedratinib because she thought Ruxo was making my anemia worse. I had another BMB in October of this year and even though my numbers are now pretty stable, (although my platelets are still high) my fibrosis is now at grade 2.

This is a very complex disease and everyone is a little different. I wish you well. I apologize if this was a confusing post.

[Steve_Essex in reply to Cja1956]

Thank you for your detailed post - not confusing at all! Will discuss with my consultant and update all with what was discussed and agreed!

[Steve_Essex]

So I’ve spoke to a haematologist at Guys today… they assured me all is within range and rightly pointed out that taking the delta between the max they’ve recently been 1050 and the lowest 620 is not an accurate reflection as it’s two extremes. In reality my platelets are around the 700-800 so a +-200 is within the band they’d expect to see a fluctuation…

I guess what made this initially concerning is the rise to 1050 and drop to 620 was within 6 months - but as Hunter rightly pointed out the fluctuation can be due to other factors going on in the body… cold.. Covid jabs… muscle soreness / pain, IBS etc…

I’ve been invited for another set of bloods which I’ll do next week… also they highlighted slightly elevated liver enzymes… which i’m pretty sure is linked to a recent month of all inclusive in Greece and catching up with friends… (busted!!) - expect a week or two detox / clean living will bring this back in check!

Will keep you all posted - thanks for your informative posts and support!

[EPguy]

Pruritus is a classic polycythemia symptom, and the trouble after showering is well known.

pubmed.ncbi.nlm.nih.gov/205...

<<Pruritus is a defining feature of polycythemia vera (PV) and is seen in approximately 40% of patients. In most cases, the pruritus is characteristically triggered by contact with water (aquagenic) at any temperature>>

So at least in that, it may suggest a Dx of PV.

I checked my CBC tests and don't find a RCM (mass) result. From this reference on PV back in 2005:

pubmed.ncbi.nlm.nih.gov/158...

<<it has become evident that RCM measurement is a cumbersome and costly test that is also suboptimal in its diagnostic accuracy. As a result, the specific procedure has been abandoned by the majority of hematologists in certain countries and instead physicians are increasingly using bone marrow histology, serum erythropoietin level, and other disease-characteristic biologic markers as diagnostic tools. >>

Maybe USA is one of the countries. I also don't see much reference to it in recent documents. You might ask your Drs about this and if there are other ways to consider your Dx. It's likely more advanced now than this 2005 article.

My Dr prefers not to distinguish ET and PV at all saying current ideas place them on a continuum. I have ~same allele as you and JAK2 only, but no pruritus so far.