Possible Richter's Transformation

stillstanding · New User Joined: 09 Jul 2009 Posts: 2

Hello All,

My husband was dx in 2003 with atypical CLL and although his wbc has been progressing steadily, he remained on W&W as he was asymptomatic. All that changed in mid-May when he developed severe pain in his upper left quadrant that took us to the ER. A CT scan revealed a massively enlarged spleen (19cm) with multiple suspicious lesions. His oncologist at Sloan-Kettering ordered a PET scan that was highly suggestive of Richter's Transformation.

The onc. indicated that the only way to get absolute proof of Richter's was with a biopsy and he would only do that after a splenectomy was done. He felt it was more important to treat the aggressive lymphoma, Richter's or not, with R-CHOP as soon as possible rather than do a splenectomy on a massively enlarged spleen and then do the chemo.

My husband has completed 4 cycles of R-CHOP and is responding very well. His blood work is normal for the first time since dx and his spleen is normal in size. However, only CT and PET scans will reveal whether the lymphoma has been eradicated or reduced. Post treatment options will be discussed after these tests are completed prior to his 5th cycle of chemo.

I'd like to know if anyone has had experience with transformed CLL; Richter's or another transformation to aggressive large cell lymphoma.

Thank you for any help offered.

Stella & Peter

menalinda · New User Joined: 08 Oct 2009 Posts: 7 Location: Germany

Hi Stella and Peter,
my mother also have a transformation from cll into richters syndrom. i'm from germany and have found this forum by google. richters syndrom is so rare, german sites are difficult to find, so i am here.

My mother had cll for 10 years, the disease was asymptomatic and she didn't need a chemo- treatment. the transformatioon came suddenly in august. Now, she is in R- CHOP treatment, yesterday she got the 2.cyclus and she is still well!!!

it is a hugh advantage, that my mum was untreated during her cll- period!!
and your husband too!! both are chemotherapie-sensitive!
do you know the richter- syndrom- score?
there are five parameters who shows the prognosis.
of course, Richters syndrom got in general a poor prognosis, but there are differences in median survival time and these differences are depending on that score parameters.

try to be hopefull! There are cases in which richters- patients live more than 2-3 years without progression!!

how old is your husband? my mum is 58 years old

Greets from germany!
menalinda

stillstanding · New User Joined: 09 Jul 2009 Posts: 2

Guten Tag Menalinda!

Thanks for responding! I'd given up hope of hearing from anyone on this site. What a coincidence that you're from Germany. My husband's CLL is familial and its origins lie in Deutchland. My husband's family is from Gersfeld and both he, his brother, and a cousin have CLL. They are all born in Germany. My husband's mother's name was Richter...isn't that unbelievable? My family is from Giessen but no CLL in the family. Smile

My husband is 56 and was previously untreated for his CLL for 6 years. The transformation happened out of nowhere. We are not familiar with the 5 parameters of prognosis or a Richter's score. Can you send more information about that?

His oncologist, who is the Chief of the Lymphoma Dept. at a major cancer center in NYC, said that the Richter's has a possibility of being cured with the R-CHOP because [b]when[/b] the transformation occurs is the most important factor. If it occurs prior to treatment, then it's not chemo-resistant and is more likely to respond. He also said that if the transformation is isolated in one location rather than systemic, it's also more likely to have a complete response. Are these two of the parameters you're referring to?

Another well-known CLL expert in NYC believes that there are two origins of Richter's determined by one's mutation status. (mutated vs. unmutated)ZAP-70 is the test that determines mutation status. Both doctors have patients who have had a one-time occurrence of Richter's and are still alive after several years. We have been in contact with three Richter's survivors who have remained in remission for 1-6 years! All three fit a similar profile: no prior treatment for CLL, isolated transformed disease in one area, all in their 50's.

My husband completed R-CHOP in August and his last CT/PET scans showed no evidence of disease. Even his CLL responded for the moment and ALL his bloodwork is within normal range for the first time since before he was diagnosed in 2003. He had virtually no side-effects from the R-CHOP and is feeling very well. As a matter of fact, 10 days after completing treatment, we went to Nova Scotia, Canada for a week and had a great time. He will have another round of CT/PET scans in November and then we will know "officially" if he's in remission.

What part of Germany are you located in? Will your mom have 6 cycles of R-CHOP? How is she doing with the R-CHOP? Please send us any information or links you have about the 5 parameters to determine a Richter's score. It sounds very interesting and we're looking for all the positive information we can find because as you know, the statistics for Richter's is depressing.

Looking forward to hearing more from you!

Stella & Peter

menalinda · New User Joined: 08 Oct 2009 Posts: 7 Location: Germany

Hi !
please contact me: tindari@gmx.net

i can't post my links here!

bis bald!

[quote="stillstanding"]Guten Tag Menalinda!

Thanks for responding! I'd given up hope of hearing from anyone on this site. What a coincidence that you're from Germany. My husband's CLL is familial and its origins lie in Deutchland. My husband's family is from Gersfeld and both he, his brother, and a cousin have CLL. They are all born in Germany. My husband's mother's name was Richter...isn't that unbelievable? My family is from Giessen but no CLL in the family. Smile

My husband is 56 and was previously untreated for his CLL for 6 years. The transformation happened out of nowhere. We are not familiar with the 5 parameters of prognosis or a Richter's score. Can you send more information about that?

His oncologist, who is the Chief of the Lymphoma Dept. at a major cancer center in NYC, said that the Richter's has a possibility of being cured with the R-CHOP because [b]when[/b] the transformation occurs is the most important factor. If it occurs prior to treatment, then it's not chemo-resistant and is more likely to respond. He also said that if the transformation is isolated in one location rather than systemic, it's also more likely to have a complete response. Are these two of the parameters you're referring to?

Another well-known CLL expert in NYC believes that there are two origins of Richter's determined by one's mutation status. (mutated vs. unmutated)ZAP-70 is the test that determines mutation status. Both doctors have patients who have had a one-time occurrence of Richter's and are still alive after several years. We have been in contact with three Richter's survivors who have remained in remission for 1-6 years! All three fit a similar profile: no prior treatment for CLL, isolated transformed disease in one area, all in their 50's.

My husband completed R-CHOP in August and his last CT/PET scans showed no evidence of disease. Even his CLL responded for the moment and ALL his bloodwork is within normal range for the first time since before he was diagnosed in 2003. He had virtually no side-effects from the R-CHOP and is feeling very well. As a matter of fact, 10 days after completing treatment, we went to Nova Scotia, Canada for a week and had a great time. He will have another round of CT/PET scans in November and then we will know "officially" if he's in remission.

What part of Germany are you located in? Will your mom have 6 cycles of R-CHOP? How is she doing with the R-CHOP? Please send us any information or links you have about the 5 parameters to determine a Richter's score. It sounds very interesting and we're looking for all the positive information we can find because as you know, the statistics for Richter's is depressing.

Looking forward to hearing more from you!

Stella & Peter[/quote]

menalinda · New User Joined: 08 Oct 2009 Posts: 7 Location: Germany

ok another try: look at this site (but it's hard to read!!)

http://jco.ascopubs.org/cgi/reprint/24/15/2343.pdf

menalinda · New User Joined: 08 Oct 2009 Posts: 7 Location: Germany

Hello Stella and Peter!
Hope you are well and thanks for responding! Got massive problems to post my thread but the important link is online- what a success!
Are you both able to speak German? It is really exciting that you are German Immigrants. And the Fact that your husband's Mother was called Richter is EVIL!

CLL- Families like in your Husband case are fortunately rare, only 5 % of all CLL- Patients got this typically genetic determination. In my Mom's Familiy there are no Cases of Blood Cancer, so it is obviously a unlucky coincidence, but her Sisters and Children (Me and my 4 Brothers) have a higher Risk to develop a CLL or another Blood Disease.

The Richter transformation is possibly cured with R-CHOP because now the CLL is high agressive (like the high agressive Non Hodgkin Lymphomas) and not indolent. Therefore hardcore Chemotherapies can attack these Cancer- Cells very well!!
But the Problem of Richter is, that it often comes back. But there are Exceptions!

"He also said that if the transformation is isolated in one location rather than systemic, it's also more likely to have a complete response"

I don't exactly know, but he has to know it! I suppose Richter Transformation is staged on the same Staging like the "normal" high agressive NHL, the Ann Arbor Staging. When the tumor is isolated and not systemic, your Husband got the first Stadium of Ann Arbor!! That is the best! But i don't exactly know if we can compare Richter Transformation with normal NHL. Please ask your oncologists- i want to know that too!!

I was happy to read that you know three Richter "Survivors"! How are they? Have they had a autologic Stem- Cell Transplantation or "only" R-CHOP- treatment? And what is about their CLL? Is she still exists? Please stay in Contact with them.These People are symbolizing our hope.

Yes I know the Risk- factors of getting Richter Transformation, but my Mother got no one of them! It is weird, I think the Doctors didn't know WHY it happens.

My Mother tolerates R-CHOP well, no heavy side- Effects till now. But she got alopezia (that's normal) and got a low grade of fatigue. She is not able to walk for a longer Time. She unfortunately lost muscles and weight, but now she weights 500 Gramms more!!

It is amazing that your husband got this physically fitness!! He is young (under 60) and younger people tolerate Chemotherapy better than older ones.

My mom have 6 Cycles of R-CHOP. She got R-CHOP -21, that means the next cycles happens after 21 Days. What about your husband? Did he get R-CHOP- 21 too?

It is fantastic, that he obviously got a complete Remission!! Rituximab is a fantastic Antibody. And R-CHOP is more effective than CHOP.

What are the plans for the Post- Chemo-Time? Will your husband get an Rituximab- Monotherapie for staying stable?

Please let me know!! I wish my mom achieves a complete Remission too.

I come from Lower Saxony (Niedersachsen) and live in Wolfsburg, where the Volkswagen is built.

Bis bald!
Menalinda

menalinda · New User Joined: 08 Oct 2009 Posts: 7 Location: Germany

Hello Stella and Peter!
How are you? Everything ok??
Menalinda

menalinda · New User Joined: 08 Oct 2009 Posts: 7 Location: Germany

Hi, it's me again!
Tell me, what's going on in the case of your husband. Did anything happenend? You unfortunately did not posting anymore. I'm so worry about that.
My mom will receive an autol. SCT next time.
menalinda

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