MDS/MPN overlap

[Stephen399b]

I was diagnosed 2 years ago with ET Jak2 +, plus PMF after a BMB. Since then I have been on HU and all of my bloods are ok, although a bit anaemic.

I have just had a 2nd BMB and it reports that I have ET or could have 'more complex disease from the MDS/MPV overlap'?

Does anyone else have this as a diagnosis or can help me understand what this is? I have next haemo appointment in 3 weeks and will get an explanation from him, but welcome any thoughts from friends 'out there'.

Thanks

Stephen

Stephen

[Helen1952]

Hi Stephen

I can’t help with your specifics but I can tell you that I have MPN/MDS overlap .

I started off with ET but about 18 months ago my bloods quite suddenly started to change .

My red cells were different shapes and sizes so I had my second BMB and it confirmed I had progressed to MDS

I am a RARS-T which is refractory anaemia with ringed sideroblasts with very high platelets they bounce around between 1200 and 1600 .

Unfortunately I am transfusion dependent as my hg keeps dropping every 3 weeks or so .

I have been assessed for a SCT but there is no pressing need at the moment .

I am very well looked after by my local and Guys .

I only take one HU a day now only to keep my whites from climbing .

There are several different types of MDS and I hope your heam will give you good information and your appointment goes well .

Please let us know how you get on

Helen

Ps I have had a Myloid Gene panel done which as well as the Jak 617v showed a mutation in SF3B111 which a good few people with MDS have

[Stephen399b in reply to Helen1952]

Thanks for this - I was not sure what MDS is, let alone the overlap.

I know I am continually anaemic with low red cell count despite iron tablets. I will ask if this is an indicator.

Stephen

[Ednama in reply to Stephen399b]

My husband was diagnosed with PMF/MDS type RARS. He was given a SCT in 2014, Unfortunately six months after the SCT he got really poorly with sepsis and his kidneys have stopped working and is dialysis dependant. However he is now disease free and has a good quality of life. Hope this helps you.

[1466 in reply to Ednama]

How old is too old for sct?

[crapaud]

Hello Stephen,

I personally had a MDS/MPN overlap (PMF).

In my case I had extremely low WBC/RBC and platlets. My condition was 'stabilised' using Pegasys injections up untill a couple of months before my SCT. Since I was Jak2 -ve and had no speen enlargement Jakavi (Ruxolitinib) was not necessary.

A SCT was undertaken 18 months after diagnosis due to the potential risks of my 'specific MDS/MF mix'.

MDS are pretty well documented on the net - however I would recommend talking to your specialist before jumping to any conclusions what so ever since there are so many types of MDS.

All the best for your upcoming challenges.

Gary

[Stephen399b in reply to crapaud]

Many thanks for your reply. I have next appointment in a couple of weeks and am just getting list of questions sorted!

Stephen

[crapaud]

Good idea about getting your questions listed!

One question that I never asked was: 'why have you chosen this line of treatment' when I was put on Pegasys.

That being said, specifically in my case, it was pretty clear that things were going to end up with a SCT pretty much 'sooner than later'. I was probably more concerned about that side of things rather than fully understanding the ongoing treatment at the early stages.

The decision to move on to a SCT was confirmed by the 'professor Pegasys JJK' in Paris - whatever short term treatment and mid-term treatment you are given, don't hesitate to ask for a second opinion if you feel the need.

Best regards

Gary

[Stephen399b in reply to crapaud]

Have you had your SCT? Hope it is working well for you.

Are you in Paris?

Best wishes

Stephen

[crapaud]

Yes I had a SCT in August 2015 (unrelated donor) - things are going pretty well now. Had some chronic liver GvHD for about a year in 2016/2017 that required Jakavi to sort out (this is not the 'standard' use for Jakavi) since high dose corticoids didn't solve the issue.

Still have some minor skin problems and aches and pains.

My treatment was in Angers and not Paris - but medicine is a small world with these types of illnesses and all the major hospitals discuss/exchange know-how. For example, when I went for my second opinion in Paris, the Professor JJK told me 'I already knew all about you and your case, even before you decided to make the appointment!' When I rang for the appointment the secretary told me 'earliest availability in 7 months!', when I replied that I was being treated by Doctor GBP at Angers I was given an appointment in 6weeks.

In theory I might have been able to be treated in Paris but was reassured that my 'home hospital' had all the necessary know-how with an excellent reputation and for my loved ones (and consequently for me) being near to home was extremely important.

Family and friends are very important with these types of illness!

Take care

Gary