Luspatercept / Lentiglobin and JAK2

[Edited, slight mistake]

Hi Andy and all other experts, Hope your all well.

Got a question for you, I'm a thalassaemia intermedia and my hgb sits arounf 7.5. I've never been transfused and my spleen sits around 23-24cm.
My haematologist is a little concerned with my last few blood tests as they are coming back as 6.5 - 7.0 so a drop of an average of .7
They are also a little concerned about the very slight enlargement of my spleen too. In any case, I've potentially been given the option to go onto the Luspatercept trials however;
Because of the hgb drop and slight spleen enlargement they have decided to do a G6PD test and a JAK2 test amongst a few others a month ago.
All tests have come back negative except for a very slight possibility of a JAK2 mutation. They have recommended I repeat just the JAK2 test a further two times to see if its a false positive.
I took my second blood test already and will have the results next week before I take the third and final test sometime in 3-4 weeks.

OK here goes;
Unless I misunderstood, my haematologists said that if a JAK2 mutation is found to be present I won't be able to go on the Luspatercept trials however both the US and EU clinical trials website don't mention any JAK2 exclusions:
https://www.clinicaltrials.gov/ct2/show/study/NCT03342404?term=ACE-536-B-THAL-002&rank=1#contacts
https://www.clinicaltrialsregister.eu/ctr-search/trial/2015-003225-33/GR

So I am totally confused as to why I might be excluded, although I could have just misunderstood them.

This brings me to the questions of:
Is Luspatercept still an option for me if the drug gets approved in a few years and my hgb holds out until then even with a JAK2 mutation..?
If not, will Lentiglobin be an option if that gets approval and I have a JAK2 mutation..?
Or are these treatments not allowed if any JAK2 mutations are found after their potential approval..?
Finally, I believe Hydroxyurea helps with reducing JAK2 mutations and a potential for hgb increase, would that be a better option to try assuming they don't let me go on the trial..?

Worse still, is a mild JAK2 (1% - 5% chance after the first test) mutation something for me to be really worried about?

I'm really hoping the next two tests are negative as all I can think about is cancer and it's really worrying me.  

Thanks Andy mate, sorry for the long post.

Im not quite sure , but it seems to me that your Doc is mixing some things.
The JAK2 mutation he mentioned is actualy only related to Ruxolitinib which is a JAK2 Inhibitor, and is suposed to dislarge the spleen and henche one may require less blood...
 (it doesnt work anyway or say the result is not worth)

Im not quite sure , but it seems to me that your Doc is mixing some things.
The JAK2 mutation he mentioned is actualy only related to Ruxolitinib which is a JAK2 Inhibitor, and is suposed to dislarge the spleen and henche one may require less blood...
 (it doesnt work anyway or say the result is not worth)

Hello Nomad, I'm not too sure I understand your post...?
The haematologists haven't yet confirmed that I cant go on the trial, they are just saying it may not be suitable.
I am assuming because they may want to try hydroxyurea instead as this inhibits JAK2 mutations at the same time as increase hgb and obviously going on Hydroxyurea means I cant take Luspatercept.
The results for the second JAK2 test comes out next week so I will know then. 
Either way the haematologists have not said I can't go on it yet. I was just wondering what would happen if they found a JAK2 mutation in the second test too, will that mean Luspatercept is out of the question even if the trials website says its fine..?

There seems to be no reason why the presence of the JAK2 gene would exclude you, especially since Luspatercept is also involved in an ongoing trial for Myeloproliferative Neoplasm, which is related to the JAK2 gene. I agree with Nomad that the doctor may be confused. As ,long as you haven't been recently treated for Myeloproliferative Neoplasm or had bhydroxyurea treatment recently, I think there would be no exclusion.

This is the link to the study for non-transfusion dependent beta thalassemia. https://clinicaltrials.gov/ct2/show/NCT03342404

Dq may I ask in which country do you live?
I'm wondering why you have never been transfused!? Whatever your condition may be, 7hb is not excusable! They should have transfused you already instead of doing all those tests and even if you have something absolutely different than Thalassaemia you shouldn't dwell on this level...
In my humble opinion this should be your first target

Dear Nomad . My son is sickle Beta plus and his age is two and half year and never transfused . His HB maintain 8 to 9 with Hydra and folic acid his weight is only ten Kg since last year but he is very energetic and active but question is that why he is not gaining weight?