Newbie with PV and questions!

[ExtraFox]

Hi everyone, new to the forum. I'm 50 and was told I have Polycythaemia Vera last week.

Probably like many of you on here, I ended up getting diagnosed with PV as a coincidence. I complained at my GP about persistent and worsening hip pain when walking. Eventually got sent for xray to check for arthritis, and blood tests to check for rheumatoid arthritis. Instead, found out I had advanced avascular necrosis (AVN) in both femoral heads - the heads have flattened because the bone inside has died, with no history of injury to account for this. A hip replacement is on the cards once the PV is under control.

GP said blood tests showed low iron and put me on iron tablets (in retrospect probably not wise) and told me to get another blood test in 6 months. Nearly didn't bother getting a second test but by then I wasn't feeling well so thought it might be helpful) I kept getting called back for more blood tests, got started on iron tablets again, finally managed to speak to a different GP who said all my recent blood tests had showed high levels of red cells, white cells and platelets and she wanted to refer me to haematology urgently, also to stop taking the iron tablets immediately.

I was given a diagnosis of JAK2 PV and began Hydroxycarbamide, Allopurinol and aspirin today. No venesection though, Haematologist says it would cause my white blood cells to rise even more in response. I was a bit surprised as from what I've read it's the go-to treatment. Has anyone else not been given venesection because of this?

My other question is, could PV have lead to the avascular necrosis? Because PV causes sludgy blood, could that have affected the blood supply to the femoral heads? I do feel like I've had some of the symptoms of PV for many years so the AVN could have had time to develop. But really it's just curiosity since there's no reversing the damage now.

Anyway that's quite enough from me! Would appreciate any feedback, this is such an interesting and helpful site.

Becky

[EPguy]

Welcome to the forum. It is a great place to learn, re-learn and share.

A post from this forum is consistent with your Dr's note on WBC:

healthunlocked.com/mpnvoice...

Did you get your HCT level result, and other results too? If not you should inquire, having access to your own records is important.

If your HCT is very high, venesection is a fast way to reduce it and its related risks. HCT is the most immediate risk factor in typical PV cases so your Dr should be watching it carefully. The HC should reduce all the blood counts, but it can take a few months to get there as was my case.

It could be your HCT is not so high and WBC is, so Dr is trying to balance them carefully.

On the Jak2, you should also ask whether they have an allele burden (AB) This how many of the jak2 cells are mutated. It can range from less than 5% to over 80%. This is worth knowing for future treatments.

At your relatively young age your Dr should also be discussing interferon therapy with you. For some this can stop progression and even reverse the disease. Two versions of this drug are Pegasys and Besremi.

[Mazcd Partner MPNVoice]

hello Becky and welcome to our forum. So sorry to hear that you have the problem with your hips, I hope that you are not in too much pain. I also hope that the information on our website is helping you to understand more about your PV mpnvoice.org.uk there is lots on there so have a look. And the lovely people on this forum will also be able to answer many of your questions. Best wishes, Maz

[ainslie]

Sorry to hear re your diagnosis, although not curable yet it’s very treatable. Probably the main priority is to check your Hct is below 45 or for female better 42/43. This reduces thrombotic risk, if it’s over 45 you need to venisect asap, your haem should know that, usually when people are diag with PV Hct is high and nearly always have several venisections to get it down, I have never heard of or experienced venisections increasing white cells, I have PV for 12 years and venisected for 11 of them. I hope that helps.

[hunter5582]

Hello and welcome. Glad you found your way here.

Low iron is common with PV. The KISS explanation f that your body is using up the available iron to make red blood cells. More iron = more RBCs. It is definitely contraindicated when you have PV. Many doctors are unaware of the specific needs of PV patients. The MPNs are very rare disorders. What you experienced being prescribed iron has happened to others with PV.

I am wondering of there was a scrambled communication with the hematologist. I have never heard of venesection elevating WBCs. It can definitely elevate platelets however. In fact, it increased my PLT by about 200K. Venesection is the common first intervention for people with PV. For low-risk PV (age<60, no cooccurring medical issues) venesection and aspirin is often the only treatment recommended. It may be that the AVN is deemed a risk factor. Perhaps that is why you are being recommended for cytoreduction.

For persons under the age of 60, Pegasys or Besremi is usually preferred to hydroxycarbamide. Some MPN Specialists now prefer Besremi/Pegasys for all PV patients. Note - The interferons are the only current treatment option that is potentially disease altering.

It seems like a reasonable supposition to make that the PV could be related to the AVN. This is something to discuss with a MPN Specialist. Note that most hematologists have little experience with MPNs. Consulting with a MPN Specialist is essential to ensure optimal MPN care. Here is a list. mpnforum.com/list-hem./

You did not indicate that you have gout. Is the allopurinol prophylaxis for a chemotherapy related hyperuricemia? HC can in fact cause renal impairment leading to gout. If you have a pre-existing gout or elevations in uric acid that would be a significant concern.

There is quite a lot to learn about PV and the treatment options. Here are a few resources you may find helpful.

About PV

legeforeningen.no/contentas...

onlinelibrary.wiley.com/doi...

About HC

drugs.com/monograph/hydroxy...

oralchemoedsheets.com/sheet...

There is plenty more out there in terms of resources. Mazcd already referred you to the MPNvoice site, which is an excellent place to start.

All the best.

[ExtraFox in reply to hunter5582]

Thank you EPguy, hunter5582, MAzcd and ainslie for such helpful and informative replies, I really appreciate it. I'll refer back to your suggestions before my next appointment and write some things down!

I'll ask again about venesection next time I see the Haematologist. Now realise it was platelets that she said would rise too high with venesection, not WBC. The two consultations I've had so far have felt rather hurried and left me feeling like I'd been pelted with info, some landed, more sailed over my head! At least I did get an MPN voice booklet, which lead me to this community.

I don't have gout, but have been put on Allopurinol for 1 month alongside the HydroxyCarbamide, to reduce the risk of kidney stones from uric acid crystals.

Interferon has not yet been mentioned as a possibility either, but Ruxolitinib (Jakavi) could the next step, as an alternative the HydroxyCarbamide if it's not effective or not tolerated well. I get the impression my Haem is rather keen to get me on this but has to go through the standard PV medication route first - no disrespect to the wonderful but underfunded NHS!