Finally had a pet scan on May 28 which showed extensive transformed disease above and below the diaphram with extensive head and neck disease, a pelvic mass and spleenomegaly. I am going for a biopsy next week to confirm diagnosis. If RT confirmed, then I will be admitted to Upenn for 5 days of intensive chemo EPOCH, since I have the tp53 and 17p mutations. My doctor has informed that epoch is rough treatment and I will ne needing blood transfusions during and after treatment. I guess if I do well she told me car-t has to be next. To say that I am bummed over this is an understatement! She’s not sure how long or if a remission with epoch is going to happen and I’m wondering if I should put my body through all the stress and pain if it’s only going to give me a few extra months.
Anyone had epoch for Richter’s here?
Renee
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First off, I am very sorry to read this-you have to be reeling with this news! Give yourself some time to process this and gather your support. It sounds like you have a very good team that will take you through each stage of this process. I have had friends that transformed and the way they made it through was focusing on the step they were dealing with at the time, and not allowing the big picture (which has to feel overwhelming) create panic. They posted about what they were experiencing and they kept their sense of humor, (which really helped with some of the boredom they faced during treatment). It was a ‘long go’ for them, but they did it and you can do this too! Trust and support💞!
The Nanjing Medical University Hospital study deployed a variation of EPOCH as a bridge to CAR-T / SCT treatment, with considerable success: Six patients (85.7%, 6/7) achieved complete remission (CR) at the end of [EPOCH] treatment.
Of note, one-half of the patients deemed at study entry to be candidates for cellular therapies were able to undergo allogeneic transplantation (n = 8) or chimeric antigen receptor T-cell therapy (n = 1), suggesting that VR-EPOCH as a bridge to cellular therapies may be a viable curative strategy for some patients. VR-EPOCH was active both in patients with clonally related and unrelated RS, and similarly in patients with and without TP53 mutation.
I don't know if going straight to cellular therapy is viable. The above articles suggest that EPOCH would be a bridge worth crossing. Best wishes for your next consultation.
Sorry to hear this news. Please remember to verify they are doing slice biopsies, and consider getting some sent to the Mayo or another site for a second opinion.
It never hurts for a second opinion in a serious diagnosis; set it up ahead of time so no delay. The doc can arrange for pathology to ship some out overnight the day of biopsy. I know not all places have the equipment to do the PCR or NGS testing in verifying Richter's, and whether or not it's clonally related, which is important to know. I know of 2 instances not counting myself where a presumptive diagnosis of RT turned out to be a non clonally related DLBCL. In my case, I had unusual cell morphology that apparently was some strange one-off.
Hi ReneeSusan and Sofia, me too -- had an RT scare this Spring wherein my hematologist suspected MZL or DLBCL and it turned out to be pseudolymphoma (Lymphocytoma Cutis), or at least they think so. Quite different presentation than yours, ReneeSusan (mine was a skin thing, no enlarged nodes or masses) but it's another example of confusion about Richters. Three different labs looked at the same skin biopsy and came to three separate conclusions. My hematologist tried to transfer my care to a lymphoma specialist and I believe that lymphoma doctor rejected the transfer, which is what led to the third lab review. There's alot of confusion out there. If you can get a second opinion to help you decide about the Epoch treatment, it might be helpful. Best wishes to you!!
I was told they would do a needle aspiration biopsy. Do I ask the doc to send out to another lab for confirmation? The SUV on my pet scan on nodes in neck was 17.5. My doc seems sure it is transformed, but of course biopsy to confirm. If non-clonally related how does that affect treatment? How does that change outcome?
Renee, you are taking your concerning news rather calmly, perhaps because you have been through this before. Our pinned post on Richter's Syndrome/Transformation, (RT) healthunlocked.com/cllsuppo... despite having a UK focus, still outlines what US centres of excellence in CLL management would recommend in your case.
It references this post,
healthunlocked.com/cllsuppo... which recommends the very helpful Richter transformation of chronic lymphocytic leukaemia: a British Society for Haematology Good Practice Paper
The first two Recommendations from that best practice paper are particularly relevant to you:
1. All patients with a clinical suspicion of transformed CLL and an SUVmax >5 should undergo PET-targeted biopsy of the most safely accessible 18F-FDG-avid site (1B).
2. A surgical excisional or incisional biopsy is strongly recommended to establish the diagnosis (1B). Where this is not possible, a core needle biopsy is an alternative (2B).
That's because a needle aspiration biopsy may miss out on capturing important cells for pathologists to examine, but sometimes that's all that can be safely managed.
In the diagnostics section of that paper, your first question is answered;
"These criteria can be subjective, and review of adequate biopsy specimens by at least two independent pathologists is desirable." M D Anderson in Texas and Mayo Clinic in Minnesota are two options for independent pathology labs experienced with diagnosing Richter's while under treatment with a BTKi drug.
With respect to your second question about treatment for non-clonally related RT, I understand that the treatment is the same as for clonally related RT, but there's a much better likelihood of achieving a cure than with clonally related RT. There's been some much needed progress on developing better treatments for RT in recent clinical trials and I would strongly recommend that you investigate your options for joining such a trial if RT is confirmed.
I’ve had the pet and many of nodes have high SUV especially in my neck. I am having a needle aspiration biopsy, by the interventional radiologist. There are several in my neck and he will decide after reviewing the pet. My spleen is enlarged and painful, can barely eat, mass in pelvis is also large and painful as are nodes in abdomen. My doc wants me to start chemo as soon as biopsy results are back. We are shooting fot June 12. I may sound calm, but I am scared. I will ask about second opinion, but not sure there is enough time.
I’m sure this is an enormous challenge, but you are really well versed in our disease, and you have an excellent team if you’re going to be treated at Penn. Trust the care you’re receiving, and if a core needle biopsy is what they’re recommending, that sounds like the option your doctor deems most feasible.
I’ll be thinking good thoughts for you. You can get through this.
Obviously we don't have the expertise to read the SUV values and accessibility of whatever node(s) they want to biopsy, but my understanding is that *the recommended* method is not a needle unless contraindicated, and that at least a second opinion is obtained. If this were me, I would be asking why they are choosing a needle aspirate. Because as AussieNeil has commented, it is not the recommended standard. However, it may not be possible to readily get one. If the reason they are doing the neck is convenience, the protocol has changed. And if the neck has the highest FDG, it likely can have slice done, depending on exactly where it is. If an internal node has a higher reading, even though it may be inconvenient, *that* apparently is the one to be used as of the 2023 recommendations. Of couse, patients unable to tolerate/not recommended to have an internal biopsy, can affect the final decision.
"Once the PET/CT is performed, a biopsy should be directed to the site of highest FDG avidity, even if this is not the most accessible site for biopsy.....Typically, a tissue biopsy completed through a core needle approach by an interventional radiologist is sufficient to make the diagnosis of RT, although an inconclusive biopsy result in the setting of a case with high clinical suspicion for RT should prompt consideration of either repeat core needle biopsy or, if feasible, excisional surgical biopsy. Occasionally, patients with RT will present with rapidly evolving cytopenias, and in that case, a bone marrow biopsy can be helpful to make the diagnosis of RT."
Also of note:
"however, the differential diagnosis in such cases also includes transformation events not classically categorized as RT, including Hodgkin transformation (HT) and plasmablastic lymphoma. Additionally, patients with CLL are at increased risk for second malignancies, including solid tumors, so this must also remain in the differential diagnosis."
So if the node(s) with the highest numbers aren't feasible to obtain a slice, a needle is used. And a second opinion is IMO always desirable. I have a great CLL specialist, and if I was ever told "we need to rule out RT" I am sure there would be no surprise when my response would be "ok, who do you recommend for a second opinion?"
So call or message the doc ahead of time, state you want second opinion specimen(s) sent out. Depending on your insurance, you may have to pay. Your insurance may pay for Mayo but not MD Andersen or Fred Hutchinson or Dana Farber, or vice versa. Call your insurance, ask if a second opinion is covered, I would be calling the facility to see if insurance was accepted and if not what my cost would be. I have a PPO that pays a percentage of even out-of-network costs, and my emergency savings can cover things like this. IDK what your situation is.
FWIW, here are the clinical trials listed in the database that mention RT.
I have Medicare and a supplemental. Will try to connect tomorrow to see if they cover a second opinion. But the doc wants to start chemo fairly soon as my spleen is quite large as are the nodes in my abdomen and pelvic mass.
It's very important to know what is causing those symptoms. Treatment for RT will also knock back your CLL, but if you have RT, it's likely you are feeling rather unwell, more so than you would be if it was just your CLL coming back. Do you have a recent LDH or B2M test results? LDH is commonly (but not always) several times the base level with RT.
Finally, have you asked for a free second opinion through the CLL Society's Expert Access program? cllsociety.wpengine.com/pro...
Ldh was over 300. I didn’t see b2m test ordered or results in my portal. Actually haven’t had that test since I was seeing Dr. Mato in 2017 and I remember my results were abnormal. With regards to the free second opinion, i would have to get all the results together and get them to the physician. Again, not enough time. I go to upenn for results of biopsy, on June 12, then direct admitted that day to start chemo that day. I assumed my doctor thinks time is of the essence here, cause the disease is pretty wide spread in my body now.
bkoffman may be able to help you arrange a second opinion in time, particularly given you have nearly 2 weeks before you get your biopsy results. It's very important that you are treated for the right lymphoma, presumably CLL or RT.
LDH of 300 isn't insane like a reading of 800 in RT case reports I have read. I had a reading of 341 last summer, from AIHA.
This sounds like what they did with SLLinColorado, and she didn't have RT. They started chemo the same day as the biopsy. She didn't start the second opinion request until after the initial biopsy, so there was a longer delay, a few days. In between they started standard chemo, I don't recall if it was R-CHOP or CHOP.
Now, IDK if that would have been the treatment used for whatever DLBCL she had. Because even non RT's that are DLBCL's may be treated the same as an RT. But on occasion someone's CLL that decides to "act like an acute leukemia" has been incorrectly diagnosed as RT. I can't say how rarely this happens, but it's not zero. I think I have read at least 1 post here where someone had an aggressively growing SLL that originally was thought to be RT.
I understand needing to treat, but if a center like the Mayo or MDAndersen is awaiting a specimen, at most the delay is what, 24 hours? It probably would need an overnight before 11am FedEx shipment, then time for it to be processed, read, reported. Maybe 36 hours?
It does sound like you are ill, and needing treatment, but if it happens to *not* be an RT, it's possible (not guaranteed, and I do not know any statistics surrounding the likelihood) certain monocolonal antibodies or other targeted treatments may be considered. Epoch is really immune suppressing therapy. I think this is the concern a few of us have. If whatever you have doesn't actually need the highly immune suppressing treatment.
And can you get the biopsy done sooner? So results are confirmed before the 12th?
OK. Please ask why they are not doing a slice biopsy, do you have something contraindicating one. Because slice biopsy seems to be really important in getting the best sample, and is considered the optimum by a number of specialists.
FWIW, a 2020 article on RT indicated of 54 suspected patients, only half had RT. There is a chart in there of the SUV values if you are interested. I know this isn't a large enough sample of patients to be overall statistically significant to a large degree, but it does indicate a number of people can look like they have RT and it actually is something else.
I think the overall statistics have changed since 2020, with the newer protocols, so please take the aforementioned with a grain of salt. Priss69 likely has better statistics, IDK if she checks here much for her name mentioned, but I think she is on an active Facebook group. You may want to check that out, I know I did when I had the "Richters Transformation" scare. I'm a member of it (it's a private group), and I just checked, people are posting so check it out.
I agree with SofiaDeo, When I had suspected RT my LDH was 270-280 and even with large bulky lymph above/below abdomen and very aggressive CLL, doctors did not feel I had RT due to low LDH but had PET scan to confirm that. There is also a member of this forum who started forum for RT patients. AussieNeil may be able to give you her name. Blessing going forward.
Please insist on surgical biopsy vs needle aspiration. After two separate needle aspirations in different nodes failed to provide enough matter to properly diagnose anything, I had a surgical biopsy that provided a definitive dx.
Sorry to read this. I had R CHOP when I had Richters and it worked brilliantly - I hope EPOCH will do the same. Ask your consultant about the Nurix trial rather than going straight to CAR T. xxx
I would look for a clinical trial rather than chemo from what you have shared here if it's confirmed RT unless it's not clonally related to the CLL. Ask them to check. It's important. No medical advice, just my opinion.
Dr. Nasta, from Upenn is advising epoch then car-t at some point. Why would she not mention a clinical trial? I was supposed to start a btk degrader trial at Penn in June until the Pet scan showed transformation. What trial should I be asking about? I am scheduled to be admitted in Penn June 12 to start chemo, so not much time.
If a doc is not participating in a particular trial, it might not be mentioned by them. I put the RT trials that are in the database above, here it is again.
Note this search has culled any trial that has the term "Richters Transformation" so not all may be relevant. Large research institutions like Mayo and MDAndersen and Dana Farber may have agreed to participate in one that isn't linked in the database yet.
It doesn't look like UPenn is a participant in any current trial for RT combo therapy with targeted agents.
Please note *the PET scan is not a final diagnostic tool for transformation diagnosis*. A *PET/CT to guide a site for biopsy" is how you get the diagnosis. If you had a plain PET, I would question it. *Since the biopsy is how one reaches the correct diagnosis, confirmation of the biopsy should be done before starting treatment. And if it is a RT, it's recommended it is tested to see if it is clonally related to the CLL or not. This information also drives the treatment decisions."
I'll note that I once got a frantic phone call from my local hem-onc, saying to come to the hospital immediately to start chemo, I had blasts in my bloodwork. I insisted on repeat bloodwork before doing anything. I didn't have blasts.
A few here have written about how they had a "suspected RT" and it turned out to be an aggressive CLL. Just because your nodes are growing rapidly, and the SUV values are elevated, is not a definitive diagnosis of an RT. It's possible it's your CLL suddenly becoming very active/growing quickly. I lit up like a Christmas tree during my initial workup, virtually all my nodes throughout my body showed activity. It wasn't until the diagnostic flow cytometry came back, that it turned out to be CLL and not Acute Lymphocytic Leukemia.
This is great, thanks for pointing this out! I see how some trials are coded specifically "R/R CLL" which wouldn't necessarily show up on a "CLL" search. I know sometimes searching "CLL/SLL" can give slightly different results too. I hadn't thought of doing a plain old RT search as the main one. Good to know.
I’m curious about your comment about blasts. My last labs showed 1% blasts and 2% reactive lymphocytes. I’ve been on Venetoclax for 5 years straight so am thinking I’m facing a relapse soon since I’m TP 53 mutated but am also aware that RT could be occurring as well, and that the Venetoclax may be slowing the progression dramatically Did they say it was a mistake?
Maybe they don't have an appropriate trial at U Penn, You have to move fast if the RT is confirmed with a biopsy as it must be. A quick 2nd opinion with a RT expert would be smart. I would need to know more, but there are many different trial. Unless the RT is not clonal related to the CLL, chemo like EPOCH does have the best results and adding other agents or different therapies all together might be better. Sorry to throw doubt, but if I had clonal related RT that was DLBCL, I would be looking at options beyond chemo to increase my odds of a good outcome. You really need a top expert if it's RT.
Dr Koffman can you recommend an RT expert? My biggest obstacle is travel. I am a caretaker for disabled spouse, that is my primary reason for staying locally with this. I live in NJ. I am requesting an excisional biopsy instead of aspiration as many folks here have shared the AB is not always reliable. I am presently on steroids to help reduce the size of my spleen and I am feeling better, so hoping I have time to get it all arranged. Please feel free to send me a chat if you prefer.
How far are you from Morristown? An entire floor of the hospital there does cancer research. I did my first trial there, with Dr. Charles Farber, and he is the doc who gave me the protocol to take back/consulted on, to my local hem-onc. Someone there *should* know of good RT specialists, they do a lot of research, go to conferences, etc. I see he is now the Medical Director of Oncology Research Network Development, so IDK how easy or fast it might be to see him. He does offer telehealth, IDK if that is possible for an initial visit.
I also see Dana Farber in Boston is doing at least 1 RT trial. IDK if that is too far.
I see that Pittsfield is a trial site for at least one RT study, from the link DriedSeaweed provided. There's one for Philadelphia, too.
With Boston and New York City having world class facilities, and New Jersey being relatively close, there hasn't been impetus for anyone in NJ to start yet another large facility IMO. You may have to find help at home, to go get optimal treatment.
Hi Renee, I was so very sorry to read your post, thats one heck of a treatment plan but it is going forward, there are things that can be tried and my message is, take your courage in both hands, take each day as it comes and the hurdles with it, as difficult as it seems right now get yourself in a positive frame of mind and have faith because apart from that there is very little that you can do other than take the advice and treatment offered of your medical team. I can well imagine that you are thinking of the ultimate but if it was me I would fight on, with a 'its not going to beat me' attitude and I hope you have family to help support you through this difficult time. I personally do not have CLL but my husband does and is on W/W however last year I was told I was in heart failure etc.etc., and I am so very thankful now for my stubborn nature because it has helped me through, not only this year but throughout the years with a stroke 2000, breast cancer 2008, ablation 2015 then a pacemaker, broken hip after a fall playing table tennis and other such 'stuff' but I'm still here and intend to remain so! We are all here to support you Renee, please let us know how you are getting on. Sending love and a very big HUGGGGGGG
Thanks so much for the encouragement. It means a lot. Yes, I am mustering my courage and holding on to my faith in God who has seen me through many health and other challenges. You are a warrior too. We don’t go down easy, it’s not in our nature. ❤️
Hi Renee, I understand the news about disease progressing can be shocking but please have faith. You will be fine as CLL treatments are advanced. May God bless you
I know that Dana Farber is doing amazing work on RT if it turns out to be that. I read through what others have said on here and agree. RCHOP is another chemo I have read about in addition to yours.
You have had such intelligent and informative replies from the folks here. I don’t know that with all the stress you’re going through at this time that you are able to push forward and request some answers. If it were me, I would certainly take all of these comments of different suggestions bulit point them and shoot them off to my doctor. I think it would give you so much more confidence in going forward with his suggestions or perhaps he will agree with you that the protocol could be changed. Either way it will make you feel better in understanding his position.
My. husband has RT. Bulky nodes above and below the diaphragm and in the neck. Failed treatment with acalibrutnib and also failed R-CHOP. Started a clinical trial at MD Anderson last year at this time and is in remission. The trial has been extended another year so we’ll keep the faith that it’s working. He is 65 - diagnosed in 2020. Message me if I can help with anything. Best of luck - it’s scary!
I saved this video a while back. Dr. Kittai goes into the advantages of Car-T for Richters and I think he suggests it’s best to have it first round rather than after other treatments.
You’re really going through the wringer of the emotional roller coaster of failed treatments, trials not timing out right and now this diagnosis. I recently posted my history and I know you responded to me. I know UPenn is a fine facility that w as one of the first with Car-T. Your skepticism and discouraging is understandable. Especially with the shock your new diagnosis provides to the psych. I certainly understand as this happened to me in January. Since that time my drs. Have been trying to keep me well enough (alive) to make it to a Bone Marrow Transplant. We had considered similar options like CAR-T, degraders but time being a factor we decided on the BMT (is Scheduled) with the Chemotherapy to get me there.
Your Chemotherapy is a different therapy from mine. Unfortunately I can not give you any relate from my own experience because of that. However, I would imagine they’ve had enough experience with their regiment to obtain successful results. It wouldn’t hurt to inquire about what they did for me. O. CHOP with Neulasta. My post has the details on what each of the capitols stand for. I just started my third cycle and numbers are great, feeling well. This is after spending a week in the hospital after the first treatment.
Again, I most certainly can empathize with the devastating nature of your diagnosis of RT! I hope you will find the resolve to keep fighting this crazy disease/cancer. With a few days of time to reflect, may you find the answers that give comfort, “I’m OK with this”.
I have those mutations and still had successful R CHOP for Richters. After a year's remission I relapsed again which they thought was a reoccurrence of RT. However, after lots of tests and biopsies they confirmed I had Prolymphocytic Leukaemia which is what I may have had the first time. Apparently RT and PLL are very similar and often cause misdiagnosis.
Jooby59 may I ask what treatment you are on now and how You’re doing? I just wrote my doctor through the patient portal to ask her to schedule me for an excisional biopsy instead after reading all the great information here. I want to be sure its RT before I put my body through a treatment like epoch. I am presently on steroids to reduce the size of my spleen and am feeling a little better. So I’m hoping we have time to get this all arranged before I start feeling really poorly.
I am on the Nurix Clinical Trial taking a BTK degrader. This is my 4th treatment plus chemotherapy in 5 years! Hopefully this current one will give me longer in remission - if/when I get there! x
HiI have everything crossed that the biopsy is not as bad as it sounds.
Having just gone through a similar shock of my CLL transforming. I know it is a shock as it another twist to our disease. I know everyone is is different but when I was told, before the consultant told me thier plan I said I wanted to go as aggressive as possible hit it hard.
Do your reading and if your chances are 50+% of any improvement its worth thinking about getting the treatment.
My R-CHOP treatment is kicking my butt and I'm not doing a great deal. I can see the improvements, so I am glad I went the way I did. I am lucky as I too have kept my sense of humour as you don't need to much energy for that
Wishing you the best of luck and we are all here to support you no matter what plan you chose best for yourself.
Thanks, I agree, we have managed to find a few laughs in all this and it does help! I am blessed to have great emotional support from my family. We have cried, laughed expressed our anger at this dreaded disease and it has helped me to get this far.
Yes that's the way, we have just been blessed with our first grandchild so that is helping.I'm ex military and my sons are good with dark humour too. So we laugh as much as we can.
Just reading through some of the comments and when they thought I had Richters several years a needle aspiration on nodes in my neck came back inconclusive so they then excised a couple of nodes in my neck and luckily they came back negative, so please don’t give up hope yet 🤞
Please call me if you need to chat. You have gotten so much support and great advice already. As we have discussed, I had 2 RT scares in the past. Both times, excisional biopsy was recommended as opposed to needle aspiration. I would just double check with your team that needle aspiration is the best choice for the most conclusive results.
I thought I was going to have an excisional biopsy, but the doctor said aspiration, I am going to write her and ask if they can do excisional biopsy instead.
I have found that the greatest way to ease the unknown is gain more information to narrow down speculation as much as possible, especially before you use the big guns. If they are necessary after the results then you can begin with full trust and not nagging doubt. When I was first diagnosed in 2010 my scans, node biopsy and bone biopsy showed some large B cells with possible RT. I was told to put a port in for chemo, which I did. But their were also small cells so a full node biopsy was requested before they would start chemo, (I was told we are still going to do Chemo, but we want to make sure of the drugs) at this point my complaint attitude changed to “something doesn’t seem right” and at my husband’s advice we called MDA to get a 2nd opinion. My biopsy showed my Ki67 at 60%. This whole process was Greek to me and I didn’t understand indolent or aggressive anything. All I felt was if it’s cancer-get rid of it, do what the doctor says. That was 2010 and MD Anderson re ran all the same tests and yes there were some large B cells present but the Ki67was at 40%. Because we could always treat when and if needed they suggested W&W. Again, my brain thought that made no sense, but I took a leap of faith and trusted them because we always had the treatment card available. You indeed may need to treat quickly, but you will have so much more confidence in the plan if you have a second or even a detailed discussion with your current team if you can’t get a second. Make a list of your questions, have someone go with you and do not begin any form of treatment without getting your questions answered. This is your body, you are the one that has control over what is done to it. If a doctor will not take a few minutes to answer questions to your satisfaction then it is like any business, find another one. You deserve and must feel comfortable with the plan. Incidentally my new MDA doc believes my original diagnosis was a mis-read. On my upcoming Aug 8th appointment I will also be having a bone biopsy. I have CLL activity that my recent PET uncovered, but the PET said not RT related. Again, we are in the information gathering stage with the treatment card now in the mix. I think RT is the scariest diagnosis any of us can face, which is exactly why you must have all the results possible before you treat. I wish you all the best!
24 months I learned my CLL had transformed to DLBCL. After 6 rounds of RCHOP and 18 radiation treatments, I have had a complete response for 19 months. Our genetic profiles are different and that probably makes a difference. That said, I am glad I went through treatment. I know median survival for Richters is discouraging. For everyone who has a poor experience, someone does well. I am an example of that. So, I would encourage you to consider treatment.
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