Quote:
Originally Posted by Logain
Bailie - first of all, thanks. The doctors have suggested to try for cyclosporin once her counts hit the 50s, and if that doesn't work - to do a transplant.
Neil - thank you so much for the detailed and informative reply.
Regarding the risks of repeated ATG, my source is this study (done in 1998). "The probability of developing a late clonal disorder was 53 +/- 10% after multiple, as compared to 34 +/- 7% after single exposure (P = 0.15)."
My wife is 29, and is healthy overall with the exception of her AA. She's not so young that a transplant is almost a no-brainer, but she's definitely far from the more dangerous ages. That's precisely why we're in this dilemma.
On one hand, you're right. ATG could help her again for a year, two years, or even seven. Or it could not. Or it could cause her tiny PNH clone to take a leap forward and then we'll find ourselves in a bigger problem.
On the other hand, a transplant could cure her. Or not. Or cure her and cause her to suffer from cGVHD that would make her life miserable.
There's just no way to know which road is the right one to take, and that is why we're so torn. It's like there's no right answer.
Which is why I included option #3 - to pray hard, no matter which course we'll eventually take. |
Hi Logain,
I am sorry that your wife relapsed after such a good response to rATG.
I wouldn't put too much weight on that paper from 1998. A more recent paper comparing horse to rabbit ATG had the 3 year clonal evolution at 21% with horse and only 14% with rabbit. Even this seems high compared to other studies that I have seen pertaining to clonal evolution.
I also wouldn't worry too much at this point about the PNH clone expanding because of the ATG treatment. I was told that having a small PNH clone is a positive clue that an immune attack is going on that will likely respond to ATG.
I am not a big fan on the option to wait-until-platelets-hit-50k-and-then-start-Cyclosporine. It may be that a transplant will be your only option if you take this approach. The longer you wait with an immune attack occurring, the more potential damage to progenitor stem cells, which means the less chance for complete recovery to normal counts with IST. I see cyclosporine alone as a bandaid. It may stop her counts from falling further, but in my opinion, it is not a robust, long term solution for someone so young.
It is interesting that your wife had rATG as her first line of treatment and responded so well! I wonder if they would try hATG this time, if you went the IST route?? Your wife is young, responded to rATG, and has a small PNH clone. The odds are in her favor to respond to a second round of ATG. However, ATG is rarely a permanent cure. So she will likely have to deal with this disease again 5 or 10 or 20 years down the road. If you have little ones at home now, this may be the less risky option as she will likely be better, faster with ATG.
A transplant, on the other hand, can be a permanent cure. However, it will be harder and riskier in the short term.
There is an excellent paper from 2012 entitled "How I treat Aplastic Anemia" by Dr P. Scheinberg and Dr N. Young that I would encourage you to read:
http://www.bloodjournal.org/content/...hecked=true#T1
Here is a quote from the paper that may provide some encouragement:
"We do not usually recommend UD HSCT on first relapse in younger patients because most will respond to further immunosuppression. Relapse alone has not been correlated to worse survival in SAA."
I'd also encourage you to check out the videos at the AA&MDSIF website, if you haven't already. They reorganized their website, but you can find them here:
https://www.pathlms.com/aamdsif
I hope I didn't muddy things more for you! Good luck with your decision!