Richter's Transformation: standardized therapy or clinical trial

[janeychen5712]

My father was diagnosed CLL 4 years ago and hasn't been treated. Recently he did a PET CT because of enlarged lmpth nodes and doctor said it's very possible he has Richter's Transformation. May I know if any one has same experience with Richters but not treated before? Could you share the treatment and how effectiveness the treatment is? My doctor at Dana Farber advised to join a R-CHOP and Venetolax clinical trial but I am not quite sure if i should join it or just pick the standardized therapy, which is R-CHOP only.Appreciate your thoughts.

[AussieNeil Partner Administrator]

Further to Spark_Plug 's reply, it is very important to confirm Richter's Transformation (RT) with a node biopsy. The PET scan should identify the best candidate for examination. A fine needle aspiration can miss the identifying transformed cells.

With respect to treatment choices, unlike CLL, RT is curable. There are two forms; de novo (unrelated to the CLL) and clonally derived from the CLL. Survival rates are much better with the de novo form.

Please talk to Dana Faber about what they consider to be the pros and cons of adding venetoclax. There's a recognised need for improvements in treatment effectiveness for RT . Venetoclax might well give your father a very important edge in beating RT if the biopsy confirms the suspected diagnosis.

Please keep us informed.

Neil

[janeychen5712 in reply to AussieNeil]

Thanks! I will talk to doctor next week asking more details about the trial.

[lindalou5 in reply to AussieNeil]

My recent visit for second opinion on my 'maybe' Richters diagnosis, the doctor said that it would be the R-Chop and then the Venetoclax. She said that I would have to go off the Acal..and I just assume from the way she said that, that I would not be taking anything for five months for my CLL. So can they give the V at the same time as the chemo treatment?

[AussieNeil Partner Administrator in reply to lindalou5]

R-CHOP will also decrease your CLL tumour burden

[flipperj in reply to lindalou5]

What were the circumstances on your RT diagnosis? Had you interrupted the Acalabrutinib?

[lindalou5 in reply to flipperj]

yes it was interupted by double mastectomy surgery..just for a week..But there is an ongoing problem with the lab studies, some saying that the breast cancer was in the lymph nodes under the arm. They took three of seven out. Right now the second opinion specialists are running more tests on the nodes with the possiblity of this being localized Richters because of the diagnosis of Diffuse large B cells (which occurred two years ago also when off the acal.) I am scheduled for pet scan this week. It is all so confusing and I just hope that the doctors can do a decent job of figuring this all out. I am known as a 'unique case' because of the breast cancer/CLL/and whatever this new thing is.

[flipperj in reply to lindalou5]

Thank you.

The same thing (excluding the breast cancer) happened to me, and after pressing my Dr., he acknowledged that they are seeing this acute situation (Richter like syndrome) frequently with patients on Acalabrutinib. Mine was morphologically consistent with DLBCL but the situation resolved with reintroduction of the BTKi.

The situation is poorly understood and the path forward for those of us affected even more so. I have had some lengthy discussions with my healthcare providers on this and I believe that at least the mechanism is beginning to be understood. You are wise to seek second opinions as we are in uncharted territory in many regards.

[janeychen5712 in reply to flipperj]

Do you mind sharing what is morphologically BLBCL? Did you do a PETCT/ biopsy to confirm whether it’s BLBCL or not?

[flipperj in reply to janeychen5712]

Pathology report confirming DLBCL. I had the usual tests, PET etc.

[AussieNeil Partner Administrator in reply to flipperj]

Flipperj, janeychen5712 and lindalou5 , I posted about the risk of incorrect diagnosis of tumour flare or Pseudo Richters following the interruption of ibrutinib 3 years ago. Disease Flare During Temporary Interruption of Ibrutinib Therapy in Patients with Chronic Lymphocytic Leukemia ( CLL ) - Pseudo Richters

healthunlocked.com/cllsuppo...

It's seen when there's an interruption of any BTKi therapy, though it's very unusual to occur during shorter interruptions for surgery. As you note, Flipperj, it resolves when the BTKi is reintroduced. I'm surprised if your doctor "acknowledged that they are seeing this acute situation (Richter like syndrome) frequently with patients on Acalabrutinib". Most importantly, it can fool pathologists not familiar with the condition, which is why anyone experiencing this scary event should have their 'hot' node pathology sample checked at a CLL centre of excellence, such as Mayo Clinic or MD Anderson in the USA to have Richter's Transformation (RT) confirmed. It does seem to occur more often with higher risk CLL, such as unmutated 11q del, though I've yet to see a paper elucidating what are considered the higher risk prognostic markers for tumour flare.

Janeychen, I'm sorry to say that your father's suspected case of RT can't be due to tumour flare due to an interruption in BTK therapy, given he is still in watch and wait.

Neil

[janeychen5712 in reply to AussieNeil]

Thanks for clarifying that. I believe the treatment in nowadays is better and we have more options .

[flipperj in reply to AussieNeil]

I am very familiar with the situation as I’ve been dealing with it for a few years. Much of what is currently known has not been made available on the internet so I will avoid engaging in any technical discussion here. I do know that mine has been described as being “more aggressive/worse than Richters” and that I am not alone in this regard.

[cajunjeff]

Assuming Richter is confirmed, I personally would choose the trial. Your dad gets all the benefits of R-CHOP plus the added pop of venetoclax. On top of that, your dad gets all the monitoring and extra care that goes along with the trial. If he becomes intolerant to the added venetoclax, they can stop it or try dose reductions.

Let’s hope it’s not Richters, but if it is there is still room for hope with either path you choose. You’re a good and smart daughter to seek information like this. I wish the best for you and your dad.

[janeychen5712 in reply to cajunjeff]

Thanks cajunjeff! What you mentioned about the pros joining the clinical trial totally makes sense to me. Thanks for sharing your preference openly with me.

[janeychen5712]

Thanks Jammin for replying. There will be a lymph node biopsy to confirm if it’s RT. I haven’t gotten chance to talk to the doctor. My concern would be if my father can tolerate the combination of 2 drugs.

[Stewie_1968]

Hi Janeychen , hope everything goes ok , sometimes you have to be your own advocate ask and ask again , how old is your father ? , I would also consider the trial you really are monitored closely Best wishes

Stewie

[janeychen5712 in reply to Stewie_1968]

He is 60 and hasn't been treated for CLL

[Bud129]

Focus on the confirmation as soon as possible. As the care giver it is a tough job and you need to be your father’s advocate and cheerleader. The biggest responsibility is to ensure things move in a very timely manner (days and weeks vs months) as RT can/will be aggressive. But confirmation is the next step.

Good luck and try not to get too far ahead of the doctors as it adds stress.

I’m a RT CLL/SLL (T17/53 del) 60 yr old who just hit “remission” at Penn but DF was on my peer review team.

Stay positive!

[janeychen5712 in reply to Bud129]

My father is also 60 yr old. Hope he will also get remission. Do you mind sharing what will be the next step? I heard patients with remission consider transplant or CART.

[Bud129 in reply to janeychen5712]

Everyone is different and you really need to stay focused on the issue at hand. My path was near worst case and truthfully I should not be on this earth, but I’m proof drugs, treatments and the doctors/medical teams are improving vastly with the number options emerging.

AussieNeil and CajunJeff gave very good inputs for majority of cases as they do their homework. They are also very good to warn folks you need the doctor’s input as they have the data and adjust the plan to the data, test results and inputs from observers ( team doctors, nurses and direct care givers) to your specific situation.

My team plan was to use R-EPOCH to CAR-T but like many treatment plans it may not be a straight line and mine was a line with sharp turns and even U turns that include neurosurgery and a brand new drug EPKINLY to save my life. I’m now finishing up CAR-T and in “remission”.

Good luck with the biopsy and again don’t get too far ahead of the data and the doctors.

[PaulSkinne in reply to janeychen5712]

I was 73 at the end of chemo, and Moffatt recommended a donor transplant while I was in complete remission. My local oncologist said it might work and he would support my decision, but said that if I was his older brother, he would recommend against it, since I was in remission and feeling well. I'm not eligible for a transplant now, since it's been so long since my chemo. I decided quality of life now was worth more to me than quantity (about a 40% chance) later. I hope this helps.

[Anita272]

praying for you and your dad! 🙏🙏🙏

[janeychen5712 in reply to Anita272]

thank you so much!

[GardenGrandma]

I am reporting on only one experience, but having that experience, I strongly recommend that your father get Richter’s confirmed and then do the trial. During a second opinion, Dr. Davids at Dana Farber recommended that Venetoclax be added to my husband’s treatment with RCHOP. My husband and his local doctor decided not to do that, because they were concerned about tolerating all the drugs. It turns out that my husband may not have had Richter’s but rather a very aggressive CLL/SLL. By the time he began on Venetoclax it was too late.

[janeychen5712 in reply to GardenGrandma]

thanks for replying me. Did you Mr husband do a biopsy to confirm if it’s Richters?

[GardenGrandma in reply to janeychen5712]

No. He had a needle biopsy of an easy to reach lymph node under his arm. It only showed CLL cells. His doctor concluded from PET scan clues that other lymph nodes would show Richter’s. That’s when I arranged for the second opinion. Dr. Davids at MGH suggested starting Venetoclax in addition to RCHOP. The local hematologist and my husband decided not to do that. So, be sure you Father has Richter’s before treatment. Everything takes so long so when the patient is making decisions, he’s feeling so bad that starting any treatment seems preferable to waiting longer.

[janeychen5712 in reply to GardenGrandma]

My father's PET CT SUV max is 17 and his doctor at Dana Farber highly suspected it's Richter's. The biopsy was scheduled a week later and will wait another week to know the result. It's just so hard when my father is feeling so bad but we have to wait for the biopsy result before can start the treatment

[PaulSkinne]

I had CLL/SLL for well over a year (treated with Calquence) before Richter's transformation occurred. Then my diagnosis was (confirmed by bone-marrow biopsies) stage 4 B cell non-Hodgkin's lymphoma. I had six rounds of R-CHOP, one every three weeks. I have been in remission for lymphoma for over two years, although I still have CLL. The chemo (particularly Adriamycin), was pretty bad, but the 100 mg/daily prednisone during the first five days after each treatment helped me feel nearly human. It did "jack" me up, making sleeping without THC nearly impossible. No option for an additional drug was offered, including consultation with my local oncologist, Florida Cancer Specialists, and second opinions from Moffatt Cancer Center. Hope this helps.

[janeychen5712 in reply to PaulSkinne]

Very helpful Paul! Thanks so much for sharing your story. How do you feel now? Does your doc mention any follow up treatment such as transplant or CART? I was wondering after the chemo plus Venetoclax treatment, how long remission people usually has and what is the next thing we could try. Appreciate your thoughts.

[PaulSkinne in reply to janeychen5712]

Please note my reply above.

[Kingfish6]

Need a confirmation/2md opinion. Assume RT results in DLBCL or ALL (?).

[janeychen5712 in reply to Kingfish6]

My dad’s doc said he needs to wait for biopsy result before start treatment