Progression to PV

[Murdoch01]

I was diagnosed with ET Jak 2 in 2017, I have not been feeling well for months now and was wondering how you would know if the ET has progressed to something else. I have a telephone consultation in the 14th of March with my Haematologist, they don’t seem to see us anymore since Covid

[hunter5582]

When I progressed from ET to PV about 8 years ago my old hematologist missed it. The MPN Specialist was able to see it just based on the CBC numbers. I did not experience any change in conditional symptoms nor experience any thrombosis. The only change was the erythrocytosis. About 7 years after the PV became evident, I did start to experience the burning/throbbing pain in my toes/feet. Aspirin took care of that problem.

You can look at your erythrocyte numbers yourself. These numbers cannot be interpreted without looking at other values too.

Major WHO criteria are as follows: Hemoglobin >16.5 g/dL in men and >16 g/dL in women, or hematocrit >49% in men and >48% in women, or red cell mass >25% above mean normal predicted value.

medscape.com/answers/205114...

Progression from ET to PV can occur; however, one thing to be aware of is Masked PV, where what appears to be ET is actually mPV.

practiceupdate.com/content/...

haematologica.org/article/v...

pubmed.ncbi.nlm.nih.gov/241...

Hopefully your hematologist is a MPN Specialist who can properly guide your MPN care.

Please let is know what you learn and how you get on.

[Murdoch01 in reply to hunter5582]

Thank you Hunter, I am still trying to get a referral to a MPN specialist

[BrookTownsend375 in reply to hunter5582]

Waiting on a Dx from my hematologist next week. Have seen the following probable dx's from my GP and Hemo ET, PV Erthythrocytosis, they have tested all genes too. Tell us how Erthythrocytosis effects your life--when do you take aspirin for it. Will let you all know when I have a definitive Dx. I am female 76. Also when I have a headache it is on the top of my head--never a real migraine--just feels sore.

[hunter5582 in reply to BrookTownsend375]

Looking over the WHO criteria you can predict the likely diagnosis if you are positive for both erythrocytosis and thrombocytosis. The consequences of erythrocytosis vary. We each present differently. Erythrocytosis causes hypervelocity and can raise your blood pressure. It can also increase your risk of thrombosis. There is also risk of microvascular symptoms. The headaches may actually be one of those.

The thrombocytosis carries risk of thrombosis, hemorrhage and microvascular symptoms. Note that the higher your platelets are the higher the risk of hemorrhage.

Do note that there is more to the issues we face than the number of blood cells. It is about how those blood cells behave. it appears they can be "extra sticky." In addition, dysregulation of the JAK-STAT pathway causes the body to make too many inflammatory cytokines. This inflammation causes many of the secondary symptoms we experience.

Do note that most hematologists lack experience with MPNs because they are so rare. One needs to see a MPN Specialist to obtain optimal MPN care. Here are some lists just in case you have not seen them.

mpnforum.com/list-hem./

pvreporter.com/mpn-speciali...

Do please let us know how things turn out,

[Lasbrisas1 in reply to BrookTownsend375]

I had this, 999 to Hospital, CEREBRAL VENOUS SINUS THROMBOSIS, BE CAREFUL. 2 BLOOD CLOTS ON MY BRAIN.

[Hopetohelp]

They told me my ET had changed to PV when my haemaglobin and red blood cells increased as well as platelets going up

[kiwibird37]

Hello,I was diagnosed with ET back in 2017 at 46 years old but have likely had it for many years before jak2+. Platelets were high and started on HU.

In late 2020 I had symptoms of very bad aching in my bones especially hands and elbows. Also. Unusually tired. After a few weeks I scheduled labs and found that my hgb went up from 16 to 18.5 in a couple months.

At that time doc started on phlebotomy 3 pts in 2 weeks which brought me down to 14 hgb.

Was then told it was probably masked PV.

I now take 1 hu every other day and usually have a therapeutic phlebotomy if my hgb goes over 15 which is about 2x a year.

Do get your lab work done soon as that can tell a lot about why you are not feeling well.

Good luck!

Barbara