Whats in a Dx?

[Uzza]

Hi Everybody,

Just a question...supposedly my Dx is ET, however, my platelets are well within in normal limits and have been within normal limits for a while, and in fact, trending down. My WCC is currently 12.48 and my differential Neutrophil count 76.1% and have been high for a long time. My Eosinophil DC is 5.4% as well. There no mention of a lobe shift for neutrophils or LDH, B12 levels, PCR or Elevated Alkaline Phosphatase. I have a positive Jak2 test. My father also had similar symptoms at about the same age.

I'm thinking my blood picture reflects more a Dx of Chronic Neutrophilic Leukemia. If so should I be more concerned about a transition to CML? Is it worth getting a second opinion?

Cheers,

Uri

[ccsial]

What is the state of your fathers disease. I suppose you could consider you might travel the same path.

I'd probably get a 2nd opinion.

[Uzza in reply to ccsial]

Unfortunately, he had a stroke and passed away in 2006.

Cheers

[Sarah_786]

Do you know what level your red cell count / hematocrit is? If they're high or on the higher end of normal, you could have an early case of polycythemia vera. I have PV and often have raised neutrophils and WCC- these are features of PV , but not ET as far as I know.

I've seen a few posts on here from people that had ET originally but changed to PV over time. I would personally get a second opinion.

Best wishes,

Sarah

[Uzza in reply to Sarah_786]

Hi Sarah,

My RCC was 4.42 on the lower side and Hematocrit also on the lower side but within range.

Cheers.

[socrates_8]

Hey Uri...

All a tad disconcerting is it not?

I made a Post recently about the likelihood of MPNs having a greater incidence than first thought from our First relations. My father also had three (3) remissions of Non-Hodgkins Lymphoma before passing away three days before his 87th... Something I had all but come to believe that the accepted wisdom thought most unlikely, and more prone to an acquired somatic blood condition...

...So firstly, and on the positive side of the ledger, still living a life approaching what many might consider a normal longevity, is very much a real possibility ...

And yes, definitely seek another opinion, in my view... There are many other tests that can be run to determine whether other types of rare blood conditions might be present etc...

We all MUST be our own best advocates always, in my view...

Best wishes Uri and please keep us informed as to your updates...

Steve

(Sydney)

[Uzza in reply to socrates_8]

Cheers Steve, thanks for the positives. However, I am quite concerned for my kids, with regard to the inheritance aspects of the condition, especially my daughter who is experiencing fatigue and increased uric acid levels

[socrates_8 in reply to Uzza]

Fatigue can have a great many causes, and as the incidence of having an MPN is so very rare... It may well be that you are worrying yourself somewhat needlessly at this early juncture...

Just keep monitoring her progress, and remember, teenagers are prone to all sorts of hormone imbalances that might persist for sometime... However, as I mentioned, fatigue can have a great many causes...

Best wishes Uri...

Steve

PS. Keep us Posted as to the results of your 2nd Opinion... (?)

PPS. Perhaps try to see Prof. Harrison if you are close enough to do so... There are few w/ her expertise in MPNs, in my view...

[Wyebird]

Is your consultant an MPN specialist? Why don’t you write all your concerns down and voice them at your next appointment. Take it from there regarding a second opinion. You must be quite fraught at the moment. Best wishes.

[Uzza in reply to Wyebird]

Hi Wyebird,

Fraught to some degree as I don't know if I should discuss the MPN inheritance ramifications with my daughter or not, at this stage at least.

Cheers,

Uri

[hunter5582]

It is baffling sometimes sorting all of this out. The are MPNs that do not fit neatly into a specific diagnostic category like ET-PV-MF-CML. Sometimes when the MPN is progressing the picture can be unclear. Since you are JAK2+ - do you know what your JAK2 Mutant Allele Burden is? If you have not had this tested, it is important information as a burden less than 50% is predictive of a milder course of the MPN. The test is called a JAK2 Mutant Allele Quantitative Analysis (or similar).

Definitely seek a second opinion - especially if the hematologist you see is not an MPN Specialist. Most hematologists only see a handful of MPNs in their entire career and do not really have the KSAs to sort out the complexities that can be involved. Here is a list of patient-recommended MPN expert docs mpnforum.com/list-hem./ ..

Hope you get some answers soon. All the best.

[Uzza]

Hunter,

This is really valuable information, thank-you so much!

Cheers,

Uri