I hope that you are well. As you know I made a Facebook group to bring all the Richter cases together. We are a few and these last weeks people with the same problem are entering. After leaving Ibrutinib for a few days for an intervention, they were found by analyzing a Richter by biopsy. The surprise is that absolutely nothing comes out in the Pet.
As you know, I was treated with the R-CHOP. There is a case where his doctor refuses to treat him as the Ricther because he thinks it is the Ibrutinib that has resulted in large cells. This doctor says that when you leave the Ibrutinib for a few days the cells become large for a few days and then they become small again. This doctor has this theory and that is why he does not intend to treat this woman.
What do you think?
Regards
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Priss69
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My understanding is that the Richter's diagnosis is only when the cells in the lymphoma are clones or related to the CLL cells.
Otherwise, it is a non-related Lymphoma (can be Hodgkin's or non-Hodgkin's), and not a Richter's transformation (of the leukemia to lymphoma).
People with CLL can get non-CLL related lymphoma, and RCHOP or R-EPOCH will still be the treatment for the lymphoma.
So, I presume the biopsy was of the lymphoma, and the clonal matching was done with the CLL cells in the bone marrow. It is a question worth asking of the doctors.
PET scan might show the presence of cancer, but it is only confirmed by a biopsy.
In Canada, they usually don't even do a PET scan as only the biopsy diagnosis is considered to be confirmatory for Richter's.
CT scan show the presence of enlarged lymph glands or lymphoma(s), and a biopsy of the gland/tumor/growth and matching that with the bone marrow (CLL cells) biopsy findings, confirms or eliminates the possibility of Richter's transformation.
You seem to be reporting the opposite. PET scan shows no cancer, while biopsy shows enlarged cells???
Enlarged B-cell - DLBCL (diffused large B-cell lymphoma) is still a type of cancer (lymphoma), which might or might not be related to CLL (leukemia).
Only when the DLBCL are related to CLL, it is considered to be a Richter's transformation, i.e., is the CLL transformed into a secondary cancer (lymphoma).
If the query is whether there can be just a temporary increase in cell size (because of stopping ibrutinib) in any lymph gland - I would first ask the doctors about the lymphoma diagnosis, and the results of the clonal match of the lymphoma biopsy to the bone marrow (CLL) biopsy.
Hope that clarifies...
Do feel free to ask if there is something I can help with further - or I missed entirely!
The issue is why some lymphoma comes out in the Pet and others do not.
In my case it had a growth rate of 90%. It took two months from when the biopsies were done until the treatment started. A week before treating me I still had no masses. I guess the first symptoms come out at six months. A leukemia specialist told me that it was the first time I had seen a case like mine in a 35-year career. A Richter without masses. But in France he thinks that I was diagnosed by chance and at the beginning of the disease.
Are you familiar with this 2014 paper from the Mayo Clinic?
ncbi.nlm.nih.gov/pmc/articl... Whether or not the DLBCL is clonally related to the CLL, it is still termed Richter's Syndrome/Transformation (RS or RT). For example, this case study report from the highly regarded American Society of Hematology (ASH) publication Blood 2017 notes "RS occurs in 2% to 10% of CLL cases: determining whether RS is clonally related (80% cases) to CLL is critical, as these cases carry poorer prognosis. Forty percent of RS cases develop before requiring CLL treatment and may have somewhat improved outcomes.
The challenge we face with the RS/RT naming is that "Richter's syndrome (RS), initially described in 1928 by Maurice Richter as a ‘reticular cell sarcoma’, was named in his honor years later by Lortholary in 1964, upon a clear recognition of a clonal transformation process [1,2]. RS is described by the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues as the development of an aggressive lymphoma arising in the background of chronic lymphocytic leukemia (CLL)."
A syndrome is "a group of symptoms which consistently occur together, or a condition characterized by a set of associated symptoms". We now know that RS can sometimes be 'de novo' and hence not be a transformation from the underlying CLL, but the symptoms are still diagnosed as RS. Further, it is probably the lack of surveillance for new cancers resulting from the CLL that is responsible for the rise of RS, hence the "WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues as the development of an aggressive lymphoma arising in the background of chronic lymphocytic leukemia (CLL)" (My emphasis).
I'm aware that CLL can come temporarily "roaring back" when people go off Ibrutinib, particularly if they haven't been on it long. Several of our members have reported lymph nodes swelling rapidly (which is scary as it may appear to be RS), but the nodes quickly shrink back when Ibrutinib is recommenced. I haven't heard of an analysis of the size of the CLL cells in these cases. Having some reputable references would be very helpful.
If you have been following the discussion Toñi R-S, Miles n-f and I have been having regarding RT diagnosed based on biopsy after ibrutinib/acalabrutinib have been on hold for three or more days, you will find this interesting. I just had a conversation with my brilliant CLL/lymphoma specialist. In all his years of practice, he told me he has never had a patient with a negative PET scan be positive for RT. He is convinced that biopsies are deemed positive for transformation to DLBCL because they look like DLBCL, but in actuality only look that way because the patient has been off ibrutinib/acalabrutinib. My physician is an active researcher and has every intention of studying this phenomenon and writing this research up for other doctors so no one has to undergo RT treatment that may not be necessary. In fact, he will likely be joining this group so he can gather more case studies to help support his hypothesis. I cannot stress enough the expertise of this wonderful physician, as he is known internationally and I believe he will be an incredibly valuable asset to this community.
this is difficult to comment because this phenomena is relatively new and there is not a lot of published evidenced about Richter's following targeted therapy. I'm not sure if we are seeing a new phenomena in these patients or that they don't genuinely have RIchter's. Whichever it is, time will certainly reveal the situation in each patient but of course, time is one thing the patient may not have. However, we don't want to see patients treated with RCHOP or even harsher regimes if they don't have DLBCL (Hodgkin's lymphoma treatment is ABVD).
I was in the audience when Dr Lamana reported that she has seen several cases of more slowly developing Richter's in patients that had been on Ibrutinib. I'll try to find my notes and see if they were PET +ve or not.
It would be good for your FB group if the Dr you mentioned would join and he would also have a cohort of patients that may be helpful for a study. Perhaps your members would also benefit from membership of this HU group too.
Wow...would be nice if my lymph nodes shrink, as I was having such a bad situation with my spleen and liver swelling pushing on my stomach. I had thought it was my stomach causing the nausea and throwing up. But, after testing at GI Associates found out my lymph nodes were now in that area.
I feel good. Joints destroyed by chemotherapy and radiotherapy. At the end of August I have a control. They will scan and pray that the Richter does not return. I do not notice masses neither by the neck nor by the armpits or English. Get back weight. I was thinking when eating Leukemia 45 kilos and now I weigh 52 kilos.
I try not to think about the disease and help other people who have been through the same thing as me.
I'm trying to find the maximum number of Richther cases so that doctors can study the cases. Doctors say they find few Richters and that is why they cannot study them. The idea is to find the maximum number of cases that are currently in treatment and can work with it.
We must study the behavior of the Richter and why it mutates. What I could see is that when Leukemia is more aggressive the Richter comes very fast.
I think that in cases of aggressive leukemia, they would have to monitor patients more often to be able to treat Richter at the beginning of the disease before it spreads throughout the body. Eliminating cells is not the same as eliminating masses throughout the body.
You are an awesome human being and a real role model. As difficult as it has been for you and having to live with the after effects of your treatments, you have the ability to reach inside yourself, offering empathic support to others as well as trying to find understanding of RT. Those are very special qualities.
I'm so glad you are feeling good and have gained back some weight.
Congratulations on your Facebook initiative - I have no doubt it will be a great resource!
There are already many doctors who are getting a lot of information out of the cases in Richter's Facebook group. There are doctors who are asking for permission to investigate the cases of Richter with the clean Pet Something poisonous we are getting out of all this
I think this is a fascinating conversation. Sepsur are you aware of this discussion and facebook group? To just throw my own thoughts into the mix, you might expect ibrutinib to possibly have some treatment effect on a Richters. So one possibility might be that the ibrutnib kept the DLBL under some control, and then it was discovered but hasn’t developed aggressively yet (presumably if Ibrtrunib is stopped the richters would at some point develop more normally if that was the case). Of course the top expert’s suggestion that this might be a temporary change due to ibrutinib can presumably be checked by a repeat biopsy.
.Venetoclax has been used with DLBL, so i think my gut feeling would be to discuss venetoclax treatment as an option with at least two top CLL experts if I was facing this issue. Because it does seem that might help to cover both issues. And for sure there is probably a value in having the initial biopsy reviewed by another pathologist at a bigger hospital. When my father had his DLBL (probably a transformation from follicular lymphoma) the top Pathologists at a top centre had a different view of his biopsy than the local one.
Ibrutinib too? Or are you staying off that? Will they be doing a repeat biopsy? I wonder if the expert trying to do a case series might want you to send her details of your dodgy biopsy too? I know it’s a right pain when doctors are “interested” in you. I’ve had those puzzle looks a couple of times! But at least this time the interest is for a positive reason ie clinically or you are not looking like Richters!
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