Need more info on ET JAK 2

[milw532]

I have read that ET JAK 2 is a blood cancer. Presently mine is being treated by a low dose aspirin daily. How does ET compare to the seriousness of leukemia or cancer generally? My doctor gives the impression that my numbers are in a treatable state and have not progressed to anything worse. I am worried that perhaps this is a more serious condition than I have been led to believe--though this site has had many positive comments from those who have lived healthy, long lives for many, many years with ET JAK 2. I am interested in any information you can share. Thank you!

[neela2020]

If we minus the word "cancer" (which really is scary) we are all pretty well here..unfortunately it is known to be progress over years ..for some it can be near to normal life for others it is not.

[milw532 in reply to neela2020]

Thank you for your kind words.

[mhos61]

Begin by taking a look at a MPN Voice. I’ve attached a link below.

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

[milw532 in reply to mhos61]

Good information. Thanks!

[Mazcd Partner MPNVoice]

Hi milw532, ET is classed as a blood cancer, or neoplasm. MPNs, myeloproliferative neoplasms used to be called MPDs myeloproliferative disorders and some haematologists do in fact still call them MPDs, and many of us on this site who were diagnosed prior to 2008 were told it was a disorder. The word neoplasm (new growth) is a term that has been used both for cancers (malignant neoplasms) and non-cancerous tumours (benign neoplasms). Because ET is characterized by uncontrolled cell growth, most haematologists and cancer organizations do classify them as a blood cancer. Whatever they are called though, remember that the symptoms and prognosis can vary widely, your specialist will advise you depending on your individual circumstances.

And on the scale of blood cancers, MPNs are right down the bottom of the scale.

Read the information we have on our website mpnvoice.org.uk, it has lots of very good information that you can trust.

Best wishes, Maz

[milw532 in reply to Mazcd]

Thank you for the information. The whole situation is quite scary. I have asked the doctor for the allele burden repeatedly and he keeps telling me that it really is an unproven indication. What supporting info might you have about this number and what do you suggest I do?

[charl17]

I have PMF. I do not find the phrase "blood cancer" very useful to describe my condition to others and to think about it myself. Science uses words and categories in a technical sense that is useful to scientists. It is more useful for patients and families to think of ET as a manageable chronic condition. The word cancer has all kinds of terrible connotations that are not helpful in coming to terms with the diagnosis.

[milw532 in reply to charl17]

Your words are so right. I like the thought you share that ET should be considered as a manageable chronic condition. I just do not want to fool myself about its seriousness.

[Cassie11]

Most people's life expectancy is not affected, and it is a treatable condition. If you are under 60 with no other medical conditions you are classed as low risk. It is scary at first but most people are able to come to terms with it and live a normal life. Wish you all the best.

[Ebot]

In terms of ‘risk’ generally I recall being told once by Prof Harrison that it was in the same ball park as being diagnosed with high blood pressure.

Given all the anxiety that appears to be generated by the word ‘cancer’ I’m beginning to wonder if we wouldn’t all be better off had the WHO not reclassified our diseases and we’d stuck with Myeloproliferative Disorders! (But then having made a successful critical illness claim, reclassification has its upsides!!)

[Threelions in reply to Ebot]

Good reply 👍

[Tylerdog1 in reply to Ebot]

Are you in the UK - my medical insurers only consider it be a “cancer” when it becomes termed MF .... I’m PV

[Ebot in reply to Tylerdog1]

Yes. And referring to Critical Illness cover.

[conno61 in reply to Ebot]

Classifying it as a cancer is advantageous on many fronts, not least it gives us in the UK access to free prescriptions and as you've stated critical illness claims.

[milw532]

Thanks for responding. Who is Prof Harrison ?

[Tylerdog1 in reply to milw532]

She is the leading specialist in the UK regarding MPNs.

[milw532 in reply to Tylerdog1]

I live in the US Does she have a fb link?

[Ebot in reply to milw532]

Check out the MPN Voice website. Essentially MPN Voice was set up by Prof Harrison and the team at Guy’s hospital. And it’s MPN Voice which monitors this Forum.

[Threelions]

I agree that the word “cancer” can be misleading in many cases.

ET for me put me in a “high risk” category because I had 2 tia prior to diagnosis and have hereditary high cholesterol and high blood pressure.

Good thing is that the immediate treatment plan given to me seems to be working fine and that, for me, means life goes on pretty much the same.

As I’ve said many times on here for me “there seems no point in worrying about things that worry won’t help “

All the best👍

[Wyebird]

Lots of info to digest and you’ll need lots of time to come terms with your diagnosis but I reckon it one of the best cancers to have. If there is a best cancer lol.

I’m Et Calr

[hunter5582]

Lots of great info in the other posts. I will add this. I was diagnosed with ET over 30 years ago. About 7 years ago it progressed to polycythemia vera. Still alive and kicking! I have lived a long rich life and continue to do so. The vast majority of the time I was on an aspirin-only protocol. I have never had any incidents of thrombosis and only mild splenomegaly. I am fortunate to have a relatively indolent form of a MPN.

It is scary to find out you have a "blood cancer." However, it is not imminently life threatening. It is helpful to think of the ET as a manageable long-term health concern that can effect your quality of life, but will not end it anytime soon. Yes, it is true that it can progress, but for most it does not.

You did not mention the driver mutation causing the ET (JAK2 - CALR - MPL - or triple negative). This is important to know. Many of us are JAK2 positive. If this is the case for you, there are some things to learn about. Your JAK2 mutant allele burden will have a bearing on symptom burden and prognosis. Broadly speaking, less than 50% is indicative of a more favorable course of the MPN. The JAK2 mutation (Janus Kinase 2) causes problems in the JAK-STAT pathway (Janus Kinase - Signal Transducer and Activator of Transcription proteins). The JAK-STAT pathway is regulates many body processes: hematopoiesis (making blood cells), cytokine production, inflammatory response, immune system, tumorigenesis, apoptosis (cell death), and more. Essentially the "on" switch is "on" too much and it makes your body make too many blood cells. It can also make your body make too many inflammatory cytokines. This overproduction of inflammatory cytokines is thought to be related to many of the secondary symptoms those with the JAK2 mutation experience.

Since you are new to the diagnosis there is lots to learn. Please give yourself some time to wrap your head around this and try not to worry too much. I find it helpful to turn the worry into action and provide the impetus to learn more. There are excellent resources, like this forum, to start with. The patient education links already provided are a great place to start. Once you have a foundation, you can look at some of the professional literature to lean more; however, unless you are a medical professional expect to do a LOT of secondary reading to understand what you are seeing.

Hope this (and the other posts) give you a good place to start on the MPN journey. All the best to you.

[Aquinas in reply to hunter5582]

My goodness 1 I found the information you have just given sooo helpful. It is the first time I have found out that JAK 2, for instance, stands for Janus Kinase 2).I am very grateful to you for your post.

[DJW1]

Hi, I was diagnosed with primary myelofibrosis in May of 2018. There are 3 forms of MPNs, which can progress (but don't always) from one to another in this order ET - PV - MF. In approximately 20% of MF cases, it can progress to leukemia, but a very serious one. You mentioned in reply to another comment that you are in the states. (I live on Vancouver Island) Are you familiar with the MPN Education Foundation? They are a tremendous resource for those with MPNs. My wife and I attended the conference that the group holds every two years at the Mayo Clinic in Scottsdale, and learned a lot. The next one is about a year away, here is a link to the last one:

mpninfo.org/conferences/201...

In terms of prognosis, have you had any genetic testing done yet? That will tell your specialist a lot about what to expect in the long term, ie stem cell procedures, etc.

Also, I really advocate going to more than one specialist, I have seen 3, plus others that have performed specialized testing. You will learn a lot more doing that.

Good luck!

David