A new nationwide study showed that the 5-year cumulative incidence of Richter transformation (RT) among patients with chronic lymphocytic leukemia (CLL) was 2.8%. Of those patients whose disease transformed, about half (49%) were treatment-naive prior to transformation.
The researchers identified 3 factors associated with increased risk for transformation: advanced Binet stage (P<.001), unmutated immunoglobulin heavy chain variable IGHV genes (P <.001), and del(17p) (P <.001).
Among those patients diagnosed with RT after initiating treatment for CLL, 53% developed it after first-line treatment, 24% after second-line treatment, and 22% after 3 or more lines.
The median overall survival among patients with CLL and RT was 3.1 years. Those patients who were previously treated for CLL had a worse overall survival than those who were treatment naive (2.8 years vs 6.1 years; P =.03). Whether this finding could be explained by a higher proportion of clonally unrelated RT among treatment-naïve patients, remain to be addressed.
I may be wrong but I seem to remember asking my consultant what are the chances of transformation to RT and I thought he said as I'm on ibrutinib (first treatment) the chances were very low even though I am 17p as ibrutinib helps stop the transformation although i might be getting mixed up with Hodgkin's lymphoma
But that was a heavily pre treated group so you’d expect less perhaps in the first line context and indeed it seems like about 1% got Richters in the first five years
Thanks for sharing Jackie. Wish we could see the original paper.
Now that I’m on the patient side of the table I hate seeing “median overall survival” stats. When one sees around 3 or 6 years ones immediate hopeless thought is that’s all I can expect to live then if that happens.
But a median survival is NOT the same as a life expectancy. And I’m sure there especially for these really acute events like getting Richters whilst it is true that 50% of folks are still dying quite early on the other 50% who make it past those 3 or 6 year points are likely to be living a whole lot longer that that. I’m guessing that Richters probably either kills you or you get cured. But the headlines in these sort of articles summarising data like this never explains that.
Hope is crucial.
If anyone reads data like this and concludes Richters is simply a death sentence that is not correct.
I’d also like to point out that the data from this study was from 2008-2016 a lifetime ago in CLL terms. My understanding is that with newer treatments just coming on steam even more recently than that those of us unfortunate enough to have such a transformation would reasonably expect outcomes to improve further.
It’d worth remembering that a five year survival rate is always at least five years out of date and then if you are trying to predict your own next five years you can make that ten! And this papers cut off was another four years ago so it’s almost 20 years out of date for what is going to matter for those of us on the forum.
We are blessed That treatment of blood cancer is evolving so fast!
One thing I couldn't help but notice here is that the median survival time of roughly 3 years for RT, while not great, seems to have gotten significantly longer in recent years.
Dr. Lamanna wouldn’t lie to you about this Sushi. Unlike CLL, Richters can be curable but it doesn’t mean everyone can be cured. There’s so many variations, types, severity and individual health factors that for some, it’s a very tough condition to overcome.
Thank you for clarifying that for me Newdawn. Sometimes i get so confused by everything with this condition. Every time i read something about rickters it throws me off edge its just so scary😢.
Yes. But look at it this way almost 15 years ago these folks were started being followed. Roughly half of them did die from their Richters but the other half were mostly cured. This is actually better than it used to be. But over that 15 years doctors are getting better and better at treating it. And new drugs are being tried. And treatment like CART or transplants. And so the number being cured is likely to go up more.
But I’m not going to lie to you either. Life is a fatal disease. None of us gets out if it Alive. Lots of people try to pretend that it’s not and live as tho we will live for ever. We have had a light switched on by having this diseas. We can choose to be paralysed by that or we can choose to LIVE the years we have and see them as precious. Knowing the chances are good we will outlive some of those we love who seem healthy now. Enjoy life! Find something great to live for (other people is a good clue) and don’t let CLL kill you already with worry. That’s not an easy task for any of us. And it’s one I still wrestle with. But let’s commit to do our best to really LIVE.
Thank you Adrian for your response i still let it get to me very much. I fear this disease with all my soul but I'm trying not to let it get to me. I'm trying to get stronger with therapy and anti depressants. I know we will not get out of here alive nobody will. It all a reminder that we will go one day and i never use to think about that every day until this diagnosis. Dr. Lamanna told me that cll is not the killer it use to be.b that people will more likely die of other comobilities.
Newdawn is right, Sushi, Dr Lammana would not lie to you. And Dr. Lammana is right. When our own CLLCanada was diagnosed with Richter’s he wrote me a note saying that it was incurable and that the prognosis was 10 months at the most. He beat it.
The ACOR CLL site had an active sub group which called themselves Team Richter’s who’s purpose was to encourage those newly diagnosed.
In addition to Chris, I met another Richter’s survivor when I went off to Canada for the Canadian CLL Conference. She has a video of an interview on patientpower.info that she did with Andrew Schorr at that conference (2012). In 2015, at the same conference, she got me thrown out of a party that we crashed and knew all of the best places to shop. No slowing her down. We are still connected on facebook. I know several others Richter’s survivors in various online groups.Statistics and available newer understanding and treatments have changed the landscape.
Go out and crash a party or two! The most fun was getting thrown out by security while the people partying bood them! LOL!
Thank you, i deeply regret saying that about Dr. Lamanna she's my cll specialist and i have to trust her with my life. She's very sweet and caring and i love her. Sometimes i say things out of fear and i need to calm down. Every one tells me including her to forget about all this and live my life but i don't know why i can't get there . I DON'T KNOW WHY😢.
Hiya I would say that it’s impossible to just forget about it! But what is possible is to find something to distract yourself. Even if it’s just something silly on Netflix at First. Watching something that makes you laugh out loud and afterwards you realise you didn’t think about CLL for that half hour. Or you go for a walk. Or take a kid out to a theme park (not a random one of course...!). Basically little bits of being able to put it aside just for a few minutes begin to exten to longer bits. But life will never be totally the same again of course not. The trick is to hang onto things to be grateful for and things to live for. It’s not easy though!
I don't know that there are very many people who just forget about it. We learn to move forward in spite of it. That is harder for some than others, and some days are harder than others. Try to not focus on "getting there" and work on moving forward in spite of it. Adrian has some good suggestions.
A Norwegian study of 2,631 CLL patients registered between 2003 and 2012 found similar results, noting that "the development of a second hematologic malignancy among patients with chronic lymphocytic leukemia (CLL) appears to be uncommon — potentially affecting 4% of patients. cancertherapyadvisor.com/ho...
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CLL SOCIETY'S ASH POSTER on our Free second opinion program plus two important papers from ASCO
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