Richter transformation after quitting ibrutinib: a diagnostic error

[Priss69]

Hi all.

The topic of the connection between Ibrutinib and the Richter transformation is getting very interesting. In the group that I have on Facebook every day people are entering that once Ibrutinib left a few days for an operation or biopsy for another movement, Richter was diagnosed. Then the Pet have come out completely clean. There are people who have biopsied him a second time after returning treatment with Ibrutinib and by surprise the Richter disappeared.

What's going on?

Here I leave a very interesting article. In our group we are already seven people with the same problem and two have been treated with the R-CHOP.

onlinelibrary.wiley.com/doi...

[PlanetaryKim]

Wow! That is incredible! Psuedo-Richter's Transformation upon temporarily halting ibrutinib - and that it disappears when ibrutinib resumed. And explains so much. Many people (myself included) have commented on what appears to be CLL progression with any interruption of ibrutinib. I certainly experienced swelling nodes within a day or 2, and lymphoma B symptoms and cytopenias shortly thereafter.

It's astounding to me that some such people have actually been given R-CHOP when really they just need to resume ibrutinib!

I halted ibrutinib permanently in March after I got fully ramped up on Venetoclax. And within a couple of days of halting ibrutinib, I had a fever that lasted 3 weeks. When I phoned my specialist, he speculated it could be RT. I was shocked and very glad I did not pursue that question further and just waited it out, since it could have resulted in misdiagnosis and unnecessary R-CHOP. Yikes. Thank you very much for sharing this article.

kim

[Priss69 in reply to PlanetaryKim]

They only did a single biopsy and treated me with the R-CHOP with a clean Pet before and after the treatment. It will never be known if he was really a Richter or not. If it is a Richter it is most likely that it will return. The curious thing is the cases that they did a biopsy after withdrawing the Ibrutinib and when they returned to supply it. I go from having Richter to not having it in a matter of days. The Pet in all cases were clean.

[PlanetaryKim in reply to Priss69]

So glad to hear all your Pet scans were clean. That's the important thing! Are you on any treatment now? ibrutinib for the CLL?

[Sepsur in reply to PlanetaryKim]

Seems to be my story Kim - thankfully Bloor wasn’t ‘fooled’ by the biopsies - PET scan 2wks later revealed no RT -I start venetoclax ramp up on Tuesday (again) 😊

[Priss69 in reply to Sepsur]

But in the end do you have Richter?

[Sepsur in reply to Priss69]

No - Not as it stands now - am I more inclined towards transformation? That I don’t know.

[PlanetaryKim in reply to Sepsur]

Great that you get to resume Venetoclax - and no RT! Yahoo! It still astounds me that this misdiagnosis is made.

[Sepsur in reply to PlanetaryKim]

In fairness, Adrian Bloor didn’t misdiagnose - he chose to ignore the diagnosis offered up by my 3 biopsies because I didn’t present as being like someone with RT 😊

[Priss69 in reply to Sepsur]

The problem for me is that they only biopsied me once, which would never be known. At the end of August I have a consultation and they will do a scan if they find anything, but at the moment I can't find any node. The only thing is the chemotherapy that has completely destroyed me

[PlanetaryKim in reply to Priss69]

I think with time your body will recover from the chemo. I hope that doesn't take too long for you Priss!

[Sushibruno in reply to PlanetaryKim]

Me too astonishing!

[Sushibruno in reply to PlanetaryKim]

I'm gonna ask dr. Lamanna about this. Im gonna make sure she explains this to me.

[Smakwater]

Sounds like good news for You Priss,

Pseudo-Richter? They make it sound better "Not".

How about Pseudo-CLL? We could all use a little Fake Cancer. Teehee.

JM

[Priss69 in reply to Smakwater]

You're right. Copy the title of the article. What do you think about this?. If Ibrutinib really causes false Richter we would be in big trouble.

[Smakwater in reply to Priss69]

I am going to have to take time to read this a bit more in depth, as I cannot really digest it with a focused take away point.

[Priss69 in reply to Smakwater]

I spoke with several hematologists on the subject and they say that for them it is new and they are impressed because it is the first time that they see Richter with Pet clean. There is a hematologist who, from the first moment he saw my case, thought that it could be due to stopping Ibrutinib, but the problem is that they did not biopsy me later and it cannot be known.

In the Facebook group there are already seven people with the same problem and we are also two people who have been treated after the Pet clean of lymphoma appeared.

[Hidden in reply to Priss69]

Do you feel you were mis diagnosed ?

[Priss69 in reply to Hidden]

I don't know, because they didn't do a second biopsy after I went back on the Ibrutinib. I had it removed three days before the biopsy. Then the Pet did it to me 7 weeks later and the Pet came out clean. Here is the doubt. I had a Kit67 of 90% and the doctors do not turn it on because I had no masses in my body.

[Smakwater in reply to Smakwater]

Ok,

I guess that I was looking for more of a resolve or confirmation of discovery somewhere in the publication or a link to a more investigative data compilation, however, I only came up two points wherein the publishers are still to ponder.

1. Caution should be taken to distinguish pseudo‐transformation from true RT in the era of targeted therapy.

2. Larger cohort studies with comprehensive molecular evaluation are needed to better characterize this unique phenomenon...

Perspective - Discovery of similarity named phenom. More investigation required to determine measures and outcomes.

At this time, I guess we accept pseudo-richter as a better diagnostic than righters transformation?

And, use this information to self advocate and inform others so that improper treatment is avoided.

Thanks for the post Priss.

[Priss69 in reply to Smakwater]

I personally think that after the biopsy and the RT results, and there are no symptoms, they would have to biopsy again once the Ibrutinib treatment has been resumed. If the Pet come out clean and with the speed that the Richter acts, it is impossible for it to be an RT, unless the Ibrutinib is slowing it down or the effects that it can produce on our body once withdrawn by some days.

There is one certain thing. All the cases that have come out clean the Pet have the same characteristics.

Biopsy for any reason other than the CLL or a node that is playing tricks on you.

No symptoms.

Ibrutinib withdrawn for a few days before biopsy.

Clean pet

[Smakwater in reply to Priss69]

I suspect that you are correct about a follow up biopsy.

We will all be anticipating the resolve protocol - "Caution should be taken to distinguish".

[Priss69 in reply to Smakwater]

When they detected it they told me that my Richter was not behaving like a normal Richter. I consulted with different hematologists and they told me that they had not seen him in his life. We have to pass this information on to our hematologists. If the patient does not have symptoms that will biopsy again after taking Ibrutinib.

[nvp815]

This is such an extremely important topic and I think this needs to be shared with all CLL patients and specialists. Having received the diagnosis of Richter's based on a biopsy, I lived with the fear and anxiety of thinking I had this dreaded disease for several days (which felt more like a lifetime). A week later, my PET scan was normal and I was told the biopsy results were likely related to the fact that I was off acalabrutinib for four days prior to the biopsy in order to prevent bleeding. In my case, my doctor will repeat the PET scan in three months to verify it is still negative. I am otherwise asymptomatic with normal labs, so at this point RT is highly unlikely. No patient should ever have to mistakenly undergo treatment for a "pseudo" disease. Thanks for sharing and spreading the word!!

Nan

[Sepsur in reply to nvp815]

Too right - and actually for my case - it was my family that was in turmoil for the 3wk gap between biopsy results consultation & PET scan result consultation. Having been left dangling with my (as they saw it) death sentence over their heads.

[nvp815 in reply to Sepsur]

So right Sepsur!! My husband and my children were distraught as well. I hope none of us ever find ourselves in this situation again. Please stay safe and healthy. Good luck with V ramp-up!!!

[Sepsur in reply to nvp815]

Thank you - let’s hope we all stay relatively well hey?

[avzuclav]

Whoa, scary stuff! Reminds me of this thread about acalabrutinib withdrawal (which has no mention of Richter's but sounds similarly scary). healthunlocked.com/cllsuppo...