MF, gastric varices, low platelets etc etc, anyone else with simlar set of problems?

[Dodders]

I feel I'm in a bit of a pickle. I've secondary MF but also have gastric varices (ongiong treatment), portal hypertension, possible splanchnic thrombosis, anaemia, low platelets and splenomegaly. I've been on ruxolotinib since end last year, now on lowest possible dose. I was in line for a transplant (donor found) but just been told because of varices etc and risk of bleeding out that's been called off. My local haem is now thinking I'll have to come off rux because platelets are 43, hgb is back down to 84 despite transfusion a few weeks ago, but my spleen is bigger than before and will presumably get a lot bigger if I stop rux. Meanwhile the splanchnic hypertension can also cause splenomegaly. Does anyone have a similar set of pathologies? Top haem at QE B'ham said I'm somewhat unusual! I feel I'm damned if I do and damned if I don't at the moment and am a bit worried about where this is all heading.

[jane13]

Hi, I also have 2ry MF and have decided not to have a transplant, do not yet need transfusions, on 10mg x2 per day Ruxo. Unfortunately I follow your line of reasoning as I guess I'll be somewhere near your situation one day.

Thoughts:

- the outcome from SCT is now poorer, but it may be still worth thinking about? Unfortunately transplant centres are keen to get good results and i am not sure if these are risk adjusted, so they may say "no"

- how about one of the new combination drugs that have been mentioned here?

I don't want to sound miserable but seems as though it's worth taking a risk and/or being a bit pushy about innovative treatment

....very best, keep us in touch

[Dodders in reply to jane13]

Hi Jane13, thanks for your reply. I understand the risks with transplant but it seems risk of AML is greater hence I was initially heading down the transplant path, and I think I would take that risk all things considered. At 55 yrs old MF has come on a bit early. Interested in your comment on transplant centres being keen to get good results but it makes sense. I feel that if varices can be treated, spleen not too big and no other concerns then I might get 'pushy' for a transplant. I am wondering about paying for a 2nd opinion; after last Monday, and today, it all seems a bit 'life or death' all of a sudden and forking out £300 or so even if it just reaffirms what I've been told might be worth it. Thanks again.

[Paul123456]

Sorry to hear about your problems.

Have you looked at Pacritinib? Does the same job as Ruxo but can be used when Platelets very low. Not sure whether being trialed in the U.K. but may be worth asking your Hem.

ncbi.nlm.nih.gov/pubmed/295...

Best wishes Paul

[Dodders in reply to Paul123456]

Hi Paul, thanks for the reply and link. I did look into pacritinib before posting and it does sound as if it might be effective. I wonder if it is NICE approved?

Thanks again.

[socrates_8 in reply to Dodders]

Hey Dodders...

Sorry to hear of your set of issues.

I was wondering about some of your comments concerning Portal hypertension, and varices. I suffered some from some bleeding early on myself until... I found out the aspirin I was taking was NOT enteric coated to protect against such problems. Since having changed to the Enteric product, I rarely have an issue at all...

Failing that, a combination therapy might well be called for as mentioned above...

As you say the Ruxolitinib does aid the spleen & other symptoms, generally speaking. However, your platelets are so low at 43. I can understand why the doc's would be loathe to increase your dosage...

Sorry I cannot provide a better response for you.

Best wishes Dodders & hopefully you will find a solution very soon. Please keep us informed.

Steve

(Sydney)

[Dodders in reply to socrates_8]

Thanks Steve, I stopped taking aspirin after a significant bleed (my hgb dropped overnight to 56) and it was this event that prompted another endoscopy and diagnosis of varices.

I am interested in what is being said about combination therapy; a combination of what in particular?

I suppose my main concern is that as each day passes I am another day closer to transition to AML and meanwhile my age and health are on a gradual downward trajectory. At some point the risk of losing my life to AML and the risk of dying from a transplant will sort of equalise, with both being high risk. However if I can get these splanchnic issues dealt with - and they should be amenable to treatment - then I can once more proceed with a transplant with the more normal 90% survival rate being the case for me.

Thanks again.

T