Richters Transformation, RCHOP+VEN?

[Lindy0311]

Hi,

I’m 42yoa and have all the bad markers (17p, TP53, etc). Unfortunately, a week ago I was officially diagnosed with RT. I started showing symptoms a month ago and the nodes in my mediastinal area of chest are already 10.5cm large. Still waiting on PET scan to see severity but biopsy already confirmed diagnosis.

I’ve spoken to two different specialist over the last two days and they both said I should start RCHOP+Venetoclax even though I have the TP53 mutation. Has anyone gone through this and had TP53? Just wondering if anyone has had any success? I’m trying to persuade them to do immunotherapy instead as my first line therapy such as P/V/O (similar to the trial at MDA); however, the specialist are hesitant because theres no published data to support it for RT yet.

Would love to hear other peoples experiences and the treatment that worked and/or didn’t work for you with RT.

[Jm954 Administrator]

Sorry to hear your SLL has transformed. R-CHOP is regarded as the standard of care for DLBCL (Richter's) around the world and gives good results but the problem is early relapse. You are fortunate to have Venetoclax added and that may help but there is little evidence at the moment I'm afraid.

Once in remission then a stem cell transplant is usually advised and your age and fitness will stand you in good stead for this. There is a specific Richter's Facebook page that you might find helpful.

Wishing you all the very best, please keep us updated.

Jackie

[Lindy0311 in reply to Jm954]

Hi Jackie, Ive been following the Richters page for a couple months now and posted my story on the Richters page as well as a cross-post for more advice. I was just checking here to see what others have experienced as not everyone uses FB and this forum is highly recommended by many.

I spoke to a stem cell team yesterday and they basically said the same thing. The goal is to reach CR after 2-3 cycles of R-CHOP+V and go straight to SCT if possible. I’m just worried that the CIT may not be the best option with the TP53 mutation.

Unfortunately, my richters is getting worst over the past two days so they are going to admit me to the hospital tomorrow to start RCHOP. And then add Ven on day 5 with a ramp up.

[Jm954 Administrator in reply to Lindy0311]

DLBCL has different markers and genetics to your CLL (even if clonally related) so I wouldn't worry too much. CLL is a low grade, slow growing lymphoma whilst DLBCL is like a racing car in comparison and will be hit hard by the chemo. As I said before, the difficulty is getting in a sustainable remission for long enough to get to transplant as it can creep back between courses of chemo. However, the Venetoclax should reduce that and I don't think your disrupted TP53 will be a problem.

Jackie

[Mystic75]

Lindy,

Although I don't have any experience with this, I wanted to wish you all the best with the treatment. Please let us know how you are doing.

D.xo

[223dogs]

Hi, I have RT, not the same markers as yourself but RCHOP is to treat the DLBCL then I am on the Stellar trial so have Acalabrutinib added on days 6 to 21 of the RCHOP cycle. Hopefully this will delay relapse. We can only hope the science, which is relatively new on the clonal nature of CLL will progress swiftly and new treatment will appear before we relapse. Everyone is different. I wish you good luck and all the best on your journey. I have my second cycle of RCHOP next week and so far I'm doing OK. I have joined the RT Facebook page and would recommend it if it's the right thing for you.

[Lindy0311 in reply to 223dogs]

I’ve already posted my story on the RT page. Did you have any major side effects fromyour first cycle? I’ve heard lot of people get a reaction to the first dose of Rituximab. How was your experience with it all?

[223dogs in reply to Lindy0311]

Yes I did react twice during the ramp up in dose, they started at 40mls in half an hour and slowly increased it but I ended up having an overnight stay in hospital so that I could have it slowly. The staff were amazing and Piriton and hydrocortisone stopped the reactions. I see the consultant on Monday and they have said they will prescribe Montelukast which apparently reduces the reaction. There is a podcast on Ribuximab on Leukaemia Care site and apparently reactions reduce with each dose. Good luck with your journey

[Jm954 Administrator in reply to 223dogs]

Well done on getting on the STELLAR Trial 223dogs. I sit on the trial management group for STELLAR and it's one of the best options available for Richter's in the UK

Jackie

[223dogs in reply to Jm954]

Thank you Jackie, that is very reassuring. I'll keep everyone posted on progress. I do feel very lucky to have got Acalabrutinib through the trial.

[Dinasantos]

🙏🙏🙏

[DoriZett]

No experience to share with your dx but sending you support and strength for whichever path you chose ahead.

[SofiaDeo]

I haven't had an RT, but have read that venetoclax is promising for TP53/del17 p folk, I have taken it for my CLL and I have a del17p on one DNA strand, the other is intact but carries a TP53 mutation. It put me in a 2 year remission, very nice.

I'm seeing trials adding it to R-CHOP and it's being added specifically to address TP53/del17p problems.

[Lindy0311 in reply to SofiaDeo]

I just did my first dose of Rituximab and had no reaction. It went bery smoothly, just a long 6-7 hours to finish the dosage. Tomorrow, I’ll start CHOP. And then on day 5 there start the Venetoclax with a ramp up to full dose. I’m also still taking Calquence to keep the CLL in check but will probably stop that after the 2nd cycle.

Did you do Gazyva (Obin) and Venetoclax? Any reaction to the venetoclax ramp up or initial dose? I’m going to ask my oncologist to switch me to Gazyva for the 2nd cycle instead of Rituximab as I’ve heard Obin is more effective.

[SofiaDeo in reply to Lindy0311]

Here's my venetoclax monotherapy journey:

healthunlocked.com/cllsuppo...

I added updates to the top of the post, so you kind of have to start at the bottom. In a nutshell, I responded immediately to the drug, but the CLL die-off was so rapid I was hospitalized longer than anticipated as a precaution, my phosphorous was rising and I was approaching changes that would have been technically TLS if things couldn't be controlled.

I felt fine besides headaches, and sleep interruption from going to the bathroom every few hours. I really pushed the oral fluids in addition to the IV ones. And voluntarily went on a low phosphorous diet, in an attempt to avoid needing medication/potential problems from elevated phosphorous. It worked. The PO4 rise leveled off before reaching the "must give meds" point.