26 with possible PV: Hello, First of I just wanted... - MPN Voice

MPN Voice

10,341 members14,253 posts

26 with possible PV

HereForAWhile profile image
35 Replies

Hello,

First of I just wanted to say thank you for taking the time to look at this if you do, I’m just very confused and not quite sure what information to take in.

For the past 6 months (maybe longer) my Hemaglobin was out of range (17.1 at its highest 18.8 then back down to 17.8 & 17.9 at my last blood test)

I’ve been passed on to a Hematology department but I just need to see some stories of people my age that do have PV living long lives - I do understand o haven’t be DX with this, but so feel it’s I coming due to my symptoms (on and off itching, sometimes collor bone ache, fatigue that comes and goes) and the high haemoglobin and hemorcrit 54%

Also my platlets were normal and white blood cell normal so I’m guessing there is a low chance (of no chance) of me having MF?

Sorry if this comes across insensitive or rude due to me not being diagnosed yet I’m just preparing myself and I also have a long wait ahead before I even see anyone (potentially a couple months)

Thanks.

Tay

Written by
HereForAWhile profile image
HereForAWhile
To view profiles and participate in discussions please or .
Read more about...
35 Replies
cmc_ufl profile image
cmc_ufl

Welcome to the forum. I know you mentioned normal platelets, but what is your exact platelet count? My guess is that if only your RBC markers are elevated, it is likely something else than PV, but it is possible to have PV even with high-normal platelets.

MF usually doesn’t present with isolated elevated RBC markers.

HereForAWhile profile image
HereForAWhile in reply to cmc_ufl

Hey cmc_ufl,

I don’t have the full set of bloods so I can’t be sure I was told they were in normal range.

I do here what your saying though, can I ask how you were diagnosed?

cmc_ufl profile image
cmc_ufl in reply to HereForAWhile

I have not been diagnosed with an MPN myself, but have been going through the diagnosis process for the last month or so. My hematologist thought I had ET due to elevated platelets (high-normal 400-450 last 10 years). My MPN specialist says it’s possible I have ET but unlikely since my JAK2/CALR/MPL tests are negative. So I’m either triple-negative, healthy with a high platelet baseline, or something else is going on.

HereForAWhile profile image
HereForAWhile in reply to cmc_ufl

Ah okay, what are the next steps for yourself then?

What have they got in mind?

Did you have any psychical symptoms while having the slightly high numbers?

cmc_ufl profile image
cmc_ufl in reply to HereForAWhile

I have never had any obvious symptoms. The next steps, if I choose to take them, are a BMB and/or further genetic testing. Specialist is not requiring them since my counts are borderline, but that will be the only way I will find out for sure. Although with my platelets not being so high, the BMB not be conclusive.

Lettie_WP profile image
Lettie_WP in reply to cmc_ufl

Hi cmc_ufl, sorry to jump in. But I'm curious as to what you said about a bmb being inconclusive. I'm under investigation for PV atm. I have high / borderline high haemoglobin and heamatocrits, but all other FBC measures normal. I am JAK2 neg, but ow EPO. I've been in this not knowing limbo for a year. I had hoped a bmb would tell me either way, but my consultant says not.

cmc_ufl profile image
cmc_ufl in reply to Lettie_WP

In my case, I’m possible ET due to chronic mildly-elevated platelets (borderline ET threshold). My specialist told me that, about half the time, a BMB for ET comes back as inconclusive, especially when platelets are high-normal like in my case. Says it likely wouldn’t justify doing such an invasive procedure since they might not be able to differentiate someone with mild ET from someone with a naturally high platelet baseline since both would likely have elevated megakaryocytes. He says I can have it if I want, but he isn’t requiring it. So I’m in limbo until further notice waiting for my bloodwork to change at some indeterminate point in the future (or not), unless I decide to go for the BMB or further genetic testing.

hunter5582 profile image
hunter5582 in reply to Lettie_WP

This is the situation where a BMB is warranted. Reference WHO diagnostic criteria for PV. mpnconnect.com/pdf/who-diag... .

It is possible the the BMB will not be conclusive. It may be that you are one of the 5% of people with PV who are JAK2 negative. A very small % of people with PV are positive for CALR. The only way to find out is to do the requisite studies. Given how rare PV is, especially JAK2 negative PV, it is very important for you to consult with a MPN Specialist. Most hematologists do not have the KSAs to manage this. Here is a list just in case you have not seen it before. mpnforum.com/list-hem./

If nothing else, it is worth contacting a MPN Specialist for a second opinion.

hunter5582 profile image
hunter5582 in reply to HereForAWhile

Side note - always get copies of all of your labs. You will need to them to review as you process what is going on. They should be available on a patient portal or by request from the lab or doctor's office.

Dovme profile image
Dovme

Hi HereForAWhile

Welcome to the Forum

Hopefully those with PV will respond to your questions but here is a link from MPN Voice with lots of information you might find useful

mpnvoice.org.uk/about-mpns/...

Wyebird profile image
Wyebird

I have ET but I’m sure many on this site with PV will give you support and advice.At the moment you are confused, upset and full of despair. All normal when you first find out that you stand a good chance of being diagnosed with an illness no- one wants.

You are now part of a team where you can let off steam, ask as many ‘silly questions as you like and in time offer advice to others.

Try and think of your glass half full.

Sending hugs

Mazcd profile image
MazcdPartnerMPNVoice

Hello Tay, welcome to our forum. We all completely understand how you are feeling at the moment, it is very confusing and scary when you are waiting for a diagnosis, wondering what the result will be and what that means for the future. I am sure hearing from the lovely people on this forum will help you, and I can see that Dovme has given you the link to our website, this will also help you.

Do you have any idea when you will be seen by a haematologist?

Best wishes, Maz

HereForAWhile profile image
HereForAWhile in reply to Mazcd

Hey maz and co!

Appreciate the kind words, I really do.

I guess I coming to terms with the fact that it all seems very up in the air if you can live a long time with PV or not? In terms of progression to MF

You have all probably been through this and I do respect that fact, but does my being diagnosed earlier in life improve life span?

Also does PV always cause scarring which leads to MF?

In terms of hematologist I was just told that I would get a referral letter sent through but she didn’t know when and it might be a while, I wouldn’t mind if I wasn’t having symptoms now I.e fatigue and itching I was having headaches but they have mostly stopped with water uptake and I also had a abdomen scan in December that showed no enlargement of liver or spleen which is good.

Any tips on diet also so I can get a leg up if necessary and or supplements people take to help, thank you all so much for listening!

Mazcd profile image
MazcdPartnerMPNVoice in reply to HereForAWhile

Hi Tay, I do hope that you get an appointment soon. With regards to your questions, you will find the information in our booklet will answer these and more

mpnvoice.org.uk/wp-content/...

A healthy balanced diet is recommended, we have help and information on our website

mpnvoice.org.uk/living-with...

With regards to supplements and vitamins, you must always discuss this with your doctor and haematologist before taking any supplements or vitamins to ensure that there aren't any contraindications with any medication you are taking.

with best wishes, Maz

hunter5582 profile image
hunter5582 in reply to HereForAWhile

Please do look at the information Mazcd referred you to. A few short answers are:

PV does not always progress to MF. It usually does not.

Many of us with PV stick to a Mediterranean or Anti-inflammatory Diet. The key is to eat healthy. We need to give our bodies the best chance possible when managing a MPN.

Regarding access to a hematologist, I am not sure what healthcare system you are in but it is true in ALL systems that patients who advocate get seen sooner. Assertive patients receive higher quality care. Passive patients do not. It is also important to see a MPN Specialist, nit just a regular hematologist (see note above).

All the best

Manouche profile image
Manouche

Yes, early diagnosis and treatment can improve your lifespan.

« Complete molecular remission in a polycythaemia vera patient 12 years after discontinuation of interferon-alpha »

researchgate.net/publicatio...

Manouche profile image
Manouche in reply to Manouche

« In two recently reported phase two studies of peg-IFN-alpha-2a in PV patients, complete molecular remissions were obtained in 7/26 (27%) of responding patients and in 14% of 35 evaluable patients [3,4]. In this case, peripheral blood taken twelve years after stopping a four year course of IFN-alpha displayed a complete molecular remission according to

consensus guidelines [8]. In our patient, it is noteworthy that a bone marrow sample in 1997

just prior to IFN-alpha discontinuation was still positive for the JAK2 V617F mutation, yet a peripheral blood sample 12 years later was negative for this mutation. Although remission unrelated to treatment remains a possibility [9], we hypothesise that IFN-alpha caused a sufficient suppression of the JAK2 V617F bearing clone to subsequently allow full expansion of normal haematopoietic elements. Although quantitation of mutated JAK2 alleles was not been described in PV patients despite persistent, although reduced, expression of the JAK2 24 performed, demonstration of a reduction of the mutated clone in the 1997 bone marrow sample compared to that at diagnosis might have provided some support for our hypothesis. Nevertheless, development of polyclonal haematopoiesis after IFN-alpha treatment has V617F mutation [10]. In cultured CD34+ cells, isolated from patients with newly diagnosed chronic myeloid leukemia (CML), treatment with IFN-alpha causes greater toxicity to primitive progenitors responsible for the maintenance of long-term cultures compared to more differentiated CML progenitors, whilst the majority of primitive Philadelphia

chromosome negative progenitors survived [11]. These findings support the possibility that IFN-alpha might sufficiently suppress the malignant clone in myeloproliferative disorders to allow re-establishment of normal hematopoiesis. In addition, an increase in absolute numbers of CD19+ and CD56+ lymphocytes in CML patients achieving a cytogenetic complete remission with IFN-alpha compared to patients with lesser responses has been noted. Thus, an alternative or complementary explanation for the delayed achievement of complete molecular remission in our patient is that, following discontinuation of IFN-alpha, eradication of the residual JAK2 V617F mutated clone was achieved by persistent immune modulatory properties of IFN-alpha. We are unaware of any similar, documented case of PV in long term complete molecular remission so many years after stopping IFN-alpha treatment with this case adding further evidence of the utility of IFN-alpha in PV patients, including those diagnosed at a relatively young age. »

core.ac.uk/download/pdf/190...

hunter5582 profile image
hunter5582

Welcome to the forum. We are glad you found your way here at this point in what may be a very long journey living with a MPN.

What you are describing is consistent with a PV diagnosis, but you will need to work with the hematologist to determine if this is primary PV or secondary to another condition. It is important to sign from the same sheet of music as the doctors does. This is the WHO diagnostic criteria for Polycythemia Vera and the other MPNs.

mpnconnect.com/pdf/who-diag... .

PV can present with elevations in only the erythrocytes, but elevation in platelets and leukocytes is fairly common. You are correct in thinking that what you describe is not consistent with MF.

It is very important that you consult with a MPN Specialist. MPNs are rare disorders and most hematologists do not have the KSAs to provide optimal treatment. Here is a list of docs with MPN expertise. mpnforum.com/list-hem./ It is worth doing whatever it takes to have a MPN Specialist on your treatment team.

I was diagnosed with ET abut 30 years ago. It progressed to PV about 7 years ago. It was likely masked PV for quite a while longer, just never detected. I have lived a rich life and at age 65 plan to continue doing so. There have been some challenges in the last couple of years, but we all face health challenges as we age. If you do have PV, you should plan to live a long life managing the PV. This journey may have some challenges, but they can be dealt with. Treatment options are improving and there are very promising options that were not available until recently.

Please stay in touch as this unfolds. Know that you are welcome here as you sort out what is going on. Do be sure to ask any questions you have. We are all here to support each other.

HereForAWhile profile image
HereForAWhile in reply to hunter5582

Cheers everyone,

I’m not sure how to even speed up seeing the haematologist or who to see a MPN specialist in the UK (I’m based in London)

Does anyone know how to go about this?

I just wish as a lot of others on here do to see the likely hood of progression in younger patients but what can you do.

Thank you for giving me the information and last thing I’ll ask is how likely could it be a secondary cause? And will they test for this also I’ve already had a clear X-ray and ultrasound in December?

HereForAWhile profile image
HereForAWhile in reply to HereForAWhile

Also last thing sorry, is that it seems a lot of people on this forum do progress to MF, so it seems a little more likely then the 15% chance they talk about, sorry to bring that up again and also if I’m being insensitive to anyone going through MF, my thoughts are with you!

in reply to HereForAWhile

Statistics are always open to interpretation. It could be that someone who's lived with an MPN which has not progressed for 20+ years, will probably have come in terms with the disease and will not be spending time in a MPN forum.

Otterfield profile image
Otterfield in reply to

Yes, in a way I am evidence for your theory. I lived well with ET for 18 years and didn't seek out an online community until my haematologist suggested I might be progressing. He later said that I wasn't (turned out he was wrong and I have a new consultant now but that's another story!). But before this, I felt reasonably well, knew what I needed to and just wanted get on with life. So there may be many like me, and many not progressing at all. How I envy them now!

Otterfield profile image
Otterfield in reply to HereForAWhile

You're not being insensitive - see my reply to Esson. Lots of others, like me, join a community when they feel they need to. In retrospect, I wish I had joined this wonderful supportive forum sooner, but then again I had a good long time just getting on with life with ET, with the chance of progression just a distant possibility. And we unlucky ones really are a minority.

HereForAWhile profile image
HereForAWhile in reply to Otterfield

I hear that! If you don’t mind me asking how did you know or feel you were progressing? And how are you know?

I hope you’re doing well!

Thank you for replying

Otterfield profile image
Otterfield in reply to HereForAWhile

I didn't know I was progressing. I had symptoms which were attributed to other things and I was diagnosed with chronic venous insufficiency (badly swollen legs) and acid reflux (cough and swollen abdomen). I was also losing weight. My blood counts were dropping but my haematologist assured me that it wasn't Myelofibrosis. When I developed breathing problems I ended up in hospital. I had dangerously low blood sodium and my blood counts had gone haywire. A different haematologist spotted the obvious and did a BMB. Now I don't have symptoms of venous insufficiency or acid reflux. My swollen abdomen turned out to be a massively enlarged spleen. Now I am much more fatigued than I used to be (I sleep for longer and don't have the stamina to keep busy). I also have joint pain but I don't know if that's related. However, Ruxolitinib has shrunk my spleen, I've gained weight and I can walk about 2 miles a day.

Bear in mind that most people don't progress though.

hunter5582 profile image
hunter5582 in reply to HereForAWhile

There are a number of fine MPN Specialists in London and nearby. Dr. Claire Harrison who is involved with MPN Voice is a name you will see a lot from others on the forum. The best approach is to see a doc based in a MPN Clinic/Center. These are usually based in major hospitals/research centers.

Testing to rule out secondary PV is an intrinsic part of the assessment. You will be doing basic lab work to look at all of this. A basic starting point it to test for the JAK2 mutation. If you are positive for JAK2, then the dx will likely end up as PV given that you meet the other criteria. If you are JAK2 positive, you will also want to know the mutant allele burden (% of hematopoietic stem cells with JAK2 mutation). Many docs will start with a bone marrow biopsy as a routine part of the assessment, but not all do.

Regarding the likelihood of secondary polycythemia, we have no way to determine that. You need to first determine JAK2 status. If you are JAK2 negative, the likelihood of secondary PV is much higher. There is not reason to wait on the JAK2 test. That can be done now.

Regarding speeding up the process, I will let others familiar with your healthcare system weigh in on that. (I live in the USA - Medicare + private insurance) It is a fundamental truth in all healthcare systems that assertive patients receive higher quality care. Passive patients do not. That includes timely access to care. People who advocate for themselves will always be more likely to receive the care they need.

Hope that helps.

HereForAWhile profile image
HereForAWhile in reply to hunter5582

Okay thanks for this very informative, not sure how I could speed up the testing of the jak2 test but might look into it!

In terms of the allele burden what does that percentage mean?

Sorry to ask so many questions and ounce again thanks for all this info it’s much appreciated always!

hunter5582 profile image
hunter5582 in reply to HereForAWhile

Asking questions is a good thing.

There are two types of tests for JAK2. Qualitative tells you YES-NO. Quantitative tells you what percentage of your hematopoietic stem cells carry the mutation. Not all of the HSCs carry the mutation (Wild-Type). People with ET tend to have the lowest allele burden. MF the highest. PV in the middle. Lower is better in terms of how MPNs present.

Persistence is the key to getting action out of any system. The JAK2 qualitative analysis is basic medical practice for anyone with your presentation. I would think your GP could order the test. Perhaps others in the UK could advise how to prompt your system to make that happen sooner rather than later.

Suggest taking Yoda's advice - "Do or do not. There is no try." Hope you get action soon.

HereForAWhile profile image
HereForAWhile in reply to hunter5582

Thanks Hunter!

Last question I have for the day (I promise haha) does starting on Pegasys or one of the non cemo drugs due to my age mean it would lessen the chance of it turning into MF?

I’m a long way of diagnosis yet but I would like to know as I’ve read inteferon drugs started early can improve prognosis and I’m wondering if that means helping it turning into MF or AML?

hunter5582 profile image
hunter5582 in reply to HereForAWhile

The evidence is that the IFNs may in fact reduce the chance of disease progression. Some people achieve hematological and molecular remission. The IFNs appear to more specifically target the mutated HSCs and can reduce mutant allele burden. The IFNs are also preferable to hydroxyurea particularly for younger people due to the potential risks of long-term HU use. IFNs are not without their own risks. The good news is that Besremi is easier to tolerate than Pegasys. It is a significant step forward in treatment options.

Feel free to keep asking questions.

HereForAWhile profile image
HereForAWhile in reply to hunter5582

Thank you so much!

I’m wondering if those options would be available to me, is Besremi available in the UK and Europe? Do you take it? do people with PV get bone pain or is that only an MF symptom?

I take comfort in knowing this forum is here and people like you all can answer questions and help.

It’s nice to know if it is PV then I should be able to live a near to normal long life!

hunter5582 profile image
hunter5582 in reply to HereForAWhile

Besremi is approved in Europe. It is very expensive, however. Others on the UK can better advise as to how to get it approved if you need it.

Bone pain does occur in PV too. Parenthetically would note that there are all sorts of inflammatory issues that can accompany PV. Deregulation of the JAK-STAT pathway also causes significant inflammation (note overproduction of inflammatory cytokines). This is why we have so many secondary symptoms. At the core, MPNs are inflammatory disorders.

You can certainly live a long rich life managing PV. I have done so. Please plan to do the same if you do have PV.

tracey13 profile image
tracey13

My husband started off with PV his Hct was 63 he had to have pints of blood removed weekly . Eventually he went on hydroxy that was fine for two year he felt so exhausted he then had a bone marrow biopsy that revealed primary MF so he's now on ruxolitanib he's doing brilliant on this medication.He is asymptomatic now and has a normal healthy life.

He was 41 when diagnosis with PV he's now 49 .

He should of had another bone marrow biopsy last year but due to covid he never got it .

He remains healthy and feels good .

Hope this helps

MPNBlog profile image
MPNBlog

Hi Tay. As others on the site have suggested I think you need to see an MPN specialist as soon as you can. With Hct of 54, if you do have PV, you need a venesection - PV patients need to keep their Hct below 45. Drink a minimum of 2 litres of water every day, and that will help also. (If you don't have PV that hydration is still good for you.) In London, Guy's and St Thomas's Hospital are world class for PV. You need to agitate for an appointment. Do what ever it takes. Ring any of the contacts from your previous appointments, ring Guy's Haematology department, send emails, write letters, ask your GP to agitate etc. Our thoughts are with you. Let us know how you get on. Best wishes.

HereForAWhile profile image
HereForAWhile

Just incase it interests anyone I managed to get hold of my blood test results and would love to see what people think is going on

So here is three tests

21 OCT 2020

FBC

Total White Cell Count - 4.96 4.00-11.00

Red blood cell count - 5.37 4.50-5.80

Heamglobin estimation 167 130-165

Packed Cell Volume 0.525 0.40-0.54

Mean Corpuscular Volume 97.7 77-95

Platelet Count 246

JAN 25 2021

Total White Cell Count - 4.88 4.00-11.00

Red blood cell count - 5.69 4.50-5.80

Heamglobin estimation 179 130-165

Packed Cell Volume 0.549 0.40-0.54

Mean Corpuscular Volume 96.4 77-95

Platelet Count 249

FEB 19 2021

Total White Cell Count - 5.41 4.00-11.00

Red blood cell count - 5.53 4.50-5.80

Heamglobin estimation 178 130-165

Packed Cell Volume 0.523 0.40-0.54

Mean Corpuscular Volume 94.5 77-95

Platelet Count 231

As you can see my levels mostly normalised by the last test except my heamglobin, it was fascinating to finally see the numbers but has left me confused, but I will say that I’m grateful more numbers aren’t out of whack and I don’t want to come across insensitive in this forum.

Any info or ideas/insight would be great!

Cheers

You may also like...

Is a normal lifespan possible for PV patients?

- with proper management people with PV can live a normal lifespan. Richard Silver et al...

ET or PV with just a slight increase in red blood cells. Is that possible

red blood cell count (5.5 to 5.8 limit for males 6) hct 47-49 (51 upper limit). So my red blood...

I have PV with no symptoms

years ago. I live in US. I am in perfect health otherwise. No heart disease , low blood pressure,...

Diagnosis update - PV - in limbo

Good news is my bloods were all at normal level. First time my haematrocit was normal for some...

Newly diagnosed with PV

contributing factor. I have just started a series of venesections to see if this helps and if not...