ET Jak 2 neg

[Windy51]

Hi what is the difference between Jak 2neg and Jak 2pos and what is Triple neg mean is one not a bad ad the other please it’s so confusing and Et and PV everyone seems too talk about PV is this the next stage from ET and all this abbreviation I haven’t got a clue what people are talking about

[socrates_8]

Hey Windy51...

Hello, I believe that you might be a fellow Aussie as well...

Yes, there is a great deal to learn concerning MPNs, but it takes time to slowly start bringing the puzzle together, so do try to stay calm and be patient w/ yourself...

If one had been diagnosed as 'Essential Thrombocythaemia' (ET), generally speaking,

usually that's based upon a number of prior discoveries. Firstly, it may have been noticed in your last Full Blood Count (FBC), that your Platelets were elevated causing some initial concern that initiated the need for some further testing etc.

Those tests, usually seek out whether or not someone is JAK2+, which simply means that you have an unspecified amount of JAK2 within your blood, and Negative (-) simply means that you don't.

JAK2 is a 'Driver' mutation, and again is generally one of the Three (3) classic known 'Driver' mutations. The other two (2) being: * MPL & * CALR

If someone has tested Negative to JAK2, and if they are suspected of being ET, then usually they would then be tested for the other Two (2) mutations.

However, when one is tested and found Negative again for the other 'Driver' mutations, they may then be determined as being 'Triple Negative'

You might find these links from MATES in Australia useful:

mpn-mate.com/mpns-what-are-...

mpn-mate.com/essential-thro...

Stay safe & well...

Steve

[Windy51 in reply to socrates_8]

Thanks Steve yes I’m from the Gold Coast and you

[Fernsong]

Yay, I'm so pleased someone else feels as I do! Well done for speaking out, Windy! I'll follow this thread with great interest!

[hunter5582]

Our friend Steve (aka socrates_8) already gave a good explanation of the acronyms we commonly use. Here are some additional details.

Essential Thrombocythemia (ET) = thombocytosis only (too many platelets). 50% have the JAK2 mutation. 25% have the CALR mutation. 2% have the MPL mutation (%s are approximate). Some have ET with no known mutation.

Polycythemia Vera (PV) - erythrocytosis (too many red blood cells). People with PV can also experience thrombocytosis and leukocytosis (too many white blood cells). 95% of people with PV have the JAK2 mutation. A very small percentage of people with PV have the CALR mutation (extremely rare). Some have PV with no known mutation.

ET does not always progress to PV, but it can (that is what did happen to me). Sometimes people have a masked PV (may be what I actually had). It looks like ET, but really is PV. PV and ET can also progress to Myelofibrosis (MF) or more rarely into Acute Myeloid Leukemia (AML). However, the vast majority of people with ET/PV do not progress this way. Monitoring your MPN status is an ongoing part of your treatment.

JAK2 = Janus Kinase 2 gene. The mutation of this gene affects hemopoietic stem cells (HSCs). Likewise the other driver mutations (CALR, MPL) in different ways, but with similar impact. HSCs are omnipotent stem cells that give rise to the lymphoid cells and myeloid cells (platelets, red and white blood cells). Basically, these mutation turn the "on-switch" on when it should not be and the body makes too many of various types of blood cells.

There are two types of known JAK2 mutations JAK2 v617f and JAK2 Exon 12. There are other JAK2 mutations under investigation and well as other potential drive mutations.

All of the driver mutations deregulate the JAK-STAT pathway, which is one of the body's kinase systems. "There are three key parts of JAK-STAT signaling: Janus kinases (JAKs), signal transducer and activator of transcription proteins (STATs), and receptors (which bind the chemical signals)." Wikipedia Deregulation of the JAK-STAT pathway is why we experience the various symptoms seen with MPNs. The JAK-STAT pathway drives hematopoiesis (blood cell production) as well as the production of inflammatory cytokines, immune response, tumorigenesis, apoptosis (cell death in a good way) and more.

As Steve indicated, there is a lot to learn if you really want to understand MPNs well enough to make informed decisions. We each cope in our own ways, I find that knowledge is power. Feeling empowered helps me cope more effectively. The good news is that we really do have time to learn more and the resources are there to learn if we choose to do so.

Hope this helps. All the best to you.