Doctor thinks I have PV

[Phoebe776]

I wrote a post a few months ago about my gp having cause for concern with my RBC.

I have nephrotic syndrome (kidney disease) have had it since I was 8 I’m now 24

I was meant to have a jak2 test back in November but clearly they never did it.

I was referred to haematologist and he called me for a phone consultation Tuesday.

He said from my bloods he can see my RBC have been so high for 3 years

He asked if I had any symptoms which I do

I’m always tired

Itching so so bad

Headaches at least 3-4 times a week

Always lightheaded makes me feel sick.

He wants me to have the jak2 test on Tuesday, and he also what’s me to have a liver, spleen and kidney ultrasound.....

I asked what he thinks it is he said he thinks I have PV.

Reading up on google has scared me, can anyone tell me about this condition

Thank you xx

[hunter5582]

Welcome to the forum. Glad you found your way here. This is a great place to get information and support.

I was diagnosed with a MPN 30 years ago. Initially presented as ET, but progressed to PV about 7 years ago. Suspect it was masked PV for much longer than that. I have been able to manage the condition successfully for quite a long time. I am currently using a phlebotomy-only protocol to keep my hematocrit <45%, which is the key to managing PV. (Note 42% target for a female).

There is a lot to learn about PV. Suggest you pace yourself and take it one step at a time. You will have plenty of time to understand this and figure out how best to approach managing the condition if you do indeed have PV. The Voices of MPN website is a really good place to start.

Here are a few factoids that my help you initially

JAK2 - Janus Kinase 2. gene. There are two known mutations of this gene, JAKsv617f and JAKs exon 12. This mutation upregulates the JAK-STAT pathway, one of the body's kinase systems. The JAK-STAT pathway is responsible for hematopoiesis (production of blood cells), immune response, hormone production, and production of inflammatory cytokines (inflammatory response). The overproduction of blood cells and inflammatory cytokines are what causes the problems we face with PV.

Step one is to find out if you have the JAK2 mutation and what your JAK2 mutant allele burden is if you do in fact have it. The allele burden is the percentage of hemopoietic stem cells (HSC) that carry the JAK2 mutation. Not all of them do. It is these mutated HSCs that cause the problem. They are the ones that the "on switch" is always on.

Step 1.1 is to follow up on the ultrasound to check on the status of internal organs. Splenomegaly is pretty common with PV. You are not reporting symptoms consistent with significant spleen enlargement, but it better to be safe than sorry.

The symptoms you report are consistent with PV, but you need to go through the diagnostic process to be sure. Part of that is ruling out secondary polycythemia. It sounds like the hematologist is on the right track to figure out what is going on. While waiting to get the diagnosis sorted out can be disconcerting, you should have an answer soon.

Most of us with PV live relatively normal life spans. PV is a condition that often can be managed successfully. There can be challenges, but they can be dealt with.

Please et us know what you find out and stay in touch,

[Wyebird]

Welcome, I have ET I’m sure people with PV will quickly respond. This sight is amazing. The tests organised are the norm. It will take ages for your head to digest your diagnosis. Meanwhile go onto MPN voice.org.uk. Maz is a tremendous asset. Are you in the UK?

[tracey13]

Try not to worry too much PV can be managed if you do have it once treatment starts you will start to feel better. There's lots of medication out there now and it's come along way.

My husband has had PV for 8 years and he lives a normal healthy life works full time he is 48 years old now.

Don't read up on Google it can be daunting.

This forum is excellent as people on here are the people who have and live with these blood cancers.

The word cancer absolutely terrifies people but it all can be managed.

Tracey

[tracey13 in reply to tracey13]

Oh I meant to mention my husband had an enlarged spleen it was 22.2 cm since starting ruxolitanib medication it's down to 14cm still a bit enlarged but I guess until his next scan he won't know if it's come down.

My husband true to cut out sugars and he's totally caffeine free in coffee and tea it made him so anxious.

If your HCT is high you will have headaches and feel so tired. Once this starts to come down it will make you feel better if this is the case with your bloods .

[Mazcd Partner MPNVoice]

hello Phoebe, and welcome to our forum. We all completely understand how you are feeling at the moment, it is quite a scary time, and I would suggest that you read the information on our website mpnvoice.org.uk, I am sure it will help you.

It will be a lot easier once you have the results of the tests, and know definitively what your diagnosis is, you can then move forward. You will see from the lovely people who contribute to this forum, there are a lot of people with PV, so you are not alone and we are all here to help and support you. Best wishes, Maz

[Phoebe776]

Thank you everyone, also I’ve just looked at my bloods, and my haemocrit says 0.534L/L. I looked it up and also says high levels could indicate PV. He is 90% sure I have it anyway.Just very anxious

[hunter5582 in reply to Phoebe776]

The anxiety is understandable. We have all been there when first diagnosed. You should have a definitive answer pretty soon. Know that if you do get PV confirmed, you will be able to deal with it. There are treatment options for PV as well as the specific symptoms you are experiencing. Treatment options are improving and there are promising things emerging in the research.

Hang in there and stay in touch.