Leukaemia Care is a UK charity so the support information is UK based. However the information about Richter's is useful, up to date and well laid out.
UPDATE: New study provides insights into the mechanisms of Richter transfomation (RT). Using single-cell genomic & transcriptomic analyses of longitudinal samples of patients with RT, this research has revealed early seeding of Richter transformation in CLL. It found dormant RT cells could be present many, many years before their final expansion and that these cells already carried the genomic alterations observed at the time of RT by whole genome sequencing. This could lead to ways to develop new strategies and therapies for treating RT.
Sorry but this is really scary. I hate these posts. It ruins my day. I know you put them up to educate people. But it terrifies me i dont know if i could handle something like this. (I didn't know there is a chance of developing rickters with the new agents)
It's scarier if you don't know the warning signs and delay having treatment. If you take the time to read the information, you'll most likely find that you are not at high risk and can relax somewhat. In the unfortunate situation where you do have poor prognostic markers, it just means that you are at higher risk, not that you will ever get Richter's Syndrome.
This earlier post by Jackie with a brief explanation of Richter's is also well worth reading:
Neil I'm sorry to react this way i just don't know how to handle anything, i want to be a stronger person. I want to be able to face cll with optimism and courage. Thank you for always answering my replys i really appreciate it. Be well🙂.
It’s utterly understandable that you would be scared. In fact maybe you need to give yourself permission to have specific periods when your job is to be a bit more scared. In general there are two reactions to the terrifying news you have cancer of any type. One is to bury your head in the sand and trust the “experts” (who depending on which hospital you go to may not be experts at all...) trying to pretend nothing is wrong is made easier for some of us because of the fact CLL is chronic and for many it doesn’t even bother them that much at the beginning (how I wish that was true of me...)
But underneath there will be anxiety maybe e anger and definitely sorrow at the loss of health this represents. If you simply try and suppress those feelings you will find they get stronger over time not weaker. This is why some of us have counsellors where we deliberately sit down and talk about how we feel.
An alternative would be to deliberately sit down and so some research so that know what’s what wirh this disease. This is the second approach: become as much of an expert as you can. What I’ve learnt is that it doesn’t matter what health system you are under YOU have to take control of your health (unless that is you have a partner or buddy who you delegate that to).
Who is going to look out for new signs in your body? You. Who is going to notice for example one area of nodes growing much quicker than the rest. Who is going to decide whether to call your doctor or leave it say six months till the next appt by which point it might be too late to save you if it is a Richters? Yep that’s right YOU.
So you are a an intelligent woman, going back and reading that leaflet carefully is a way for you to face your fears but then also feel a sense of mastery over them. You can feel “I got this!” And then realise that it’s not that common this will happen to you but if it does it is well worth knowing enough to grab it.
Honestly we should do a study sometime of how long people on this forum live compared to similar matches CLLers who aren’t on here. I suspect we might live longer because of all the nagging to go to hospital if we have significant signs of infection (notice for example that one of the signs of Richers is a High temp so if we high tailed it down to A and E / ER because our temp was over 38s100.4 it is possible that an astute doctor might have picked up a Richters was the cause. One more reason to ideally go to an A and E which has blood cancer expert doctors on call there (UCLH in Euston is where I go for example...)
Thank you so very much Adrian. I'm happy i found this site without it and without all the smart people on here that give the best possible advice i don't know where i would be. 🙂
Hi there Sushibruno, I'm sorry if this has rocked your day
I bet you're stronger than you know but that doesn't mean you're not scared of this, I know I am and that's why I read about it.
As AussieNeil says, even though it's very unlikely to happen to any one of us, it's important to be aware of the signs and symptoms so that we can get prompt treatment.
Thank you Jackie i know that these posts are put up so that we can be more aware of what can happen and we can take action asap. i sometimes let my subconscious mind take over me. I appreciate everyone on this forum and am happy i found it.
This overall risk of 10-15% is always annoying to read. I do not find it helpful. If specific markers can make us inclined to RT then different people have vastly different "% risk".
Exactly, but per the article Jm954 referenced, we are still trying to determine which markers are responsible for an increased risk of RS/RT. Even then, many of us would find it difficult, if not impossible to have the relevant testing done. Most of us outside of the USA find it very hard to get IgHV mutation status tested. It took me 10 years to find out mine and that was only because of a donation to support testing for those approaching treatment in Australia. Notch1 is even harder to get tested and I suspect that would apply for many in the USA also. Then there's the remaining uncertainty of whether or not specific treatments increase the risk. At least we can familiarise ourselves with the symptoms and be proactive to improve our outcome. RT/RS is at least curable.
Luckily I knew from my reading that if you are tri 12 you might have Notch 1. When got the tri 12 diagnosis I knew to request a Notch test. Thats why we check this site!
ASH abstracts for this year are out. If you enter Richter's into the search engine ash.confex.com/ash/2019/web..., you'll find some quite encouraging research.
Thanks Jackie. The interesting thing for my case is that I never had any of the listed symptoms until the 4th month when my neck muscle started swelling up and my neurologist performed a lumbar puncture due to CNS issues I suffered. That was when a PET scan was ordered and with the findings my Hematologist put me in the hospital for a week to run diagnostic tests and give me chemo. Then I was immediately started on R-CHOP.
your case is so unusual in many ways, the fact that you didn't have any of the common symptoms of transformation doesn't surprise me.
Lymphoproliferative disease is most often seen in people with immune deficiency or dysregulation, quite a rare thing. Then to have such extensive CNS disease, with all the horrible pain and symptoms that you had, is very rare too. Add to that the chance of Richters (about 5%) and you can see that you are a very rare patient. The genetics of your lymphoproliferative and high grade lymphoma would be very interesting. Don't be surprised if your Dr asks for your permission to publish your case study (it will be annonymous) so that others can learn from it.
I'm sorry the transplant hasn't been successful but I am hoping the CAR-T will kick this into the grass at long last.
It’s interesting that you said my Dr might ask permission to write a case study on me. My wife, a nurse, has been saying the same thing for quite some time. When I’ve asked my Hematologist a question about my case he answered “There is nothing normal about your case!”. Well, I hope I continue the unusual streak and live a very long time!
Research recommends when a lymph node biopsy should be considered to rule out Richter transformation in CLL patients treated with ibrutinib
In this paper, Patient characteristics that predict Richter's Transformation in patients with CLL treated with ibrutinib, which includes highly respected CLL specialists among its authors, they state "A lymph node biopsy to rule out RT should be considered in patients who received ibrutinib who progress on treatment, have an elevated LDH, or progress with lymphadenopathy without lymphocytosis."
Note in associated news, that when you stop BTK inhibitor drugs like ibrutinib, even after you have been on them for years, it's possible to experience tumour flare, where lymph nodes can rapidly enlarge. This Pseudo Richter's Syndrome behaviour is frightening and good expertise is required to rule out true Richter's. Unfortunately, some people have even gone through unnecessary treatment for Richter's. Resuming the BTKi drug usually results in the tumour flare reversing.
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