It is important to know all you can about your enemy
Most of us who have been around the CLL scene for a couple of years have heard of Richter’s syndrome. Development of Richter’s syndrome carries with it ominous risks and it is a subject we cannot ignore. Early and accurate diagnosis followed by prompt treatment greatly improves chances of survival. One more case where what you (and your oncologist) do not know can kill you.
Below are the abstracts of two state-of-the-art papers from very credible researchers, published in the last week or so. I found some of the comments a little startling and I would like to share them with you. They also shed light on profiles of patients who are more at risk of developing Richter’s syndrome. If you want to read the full text of these articles send me a personal email and I will try to help you locate them.
What is Richter’s syndrome?
CLL and some types of NHL are called indolent leukemia and lymphoma respectively. In other words, both of these cancers are slow growing and most patients have one or more years before they need therapy. Under a microscope the cancerous B-cells in both cases are seen to be small and rather non-descript, with clear cell boundaries.
Both the clinical progression of CLL (slow) and the appearance of the cells (small, well defined cells) set it apart from a far more dangerous and fast growing lymphoma called DLBCL (diffuse, large B-cell lymphoma). As the acronym points out, under a microscope the cell type of DLBCL is seen to be large B-cells with diffuse cell boundaries. It is still B-cells that are cancerous, but DLBCL is a very different from CLL. This one has fangs and it is a very dangerous beast. When a patient with CLL starts showing symptoms of aggressive lymphoma – usually DLBCL, he is said to have undergone Richter’s transformation – named after Maurice Richter who first described this in 1928.
What causes Richter’s transformation?
The frank answer is that we do not yet know.
There are two ways in which DLBCL can happen. First, it can show up on the scene as an entirely new cancer. By now you should know that CLL patients are more at risk of getting secondary cancers. Skin cancer is a prime example, but it is by no means the only secondary cancer that can happen as a result of the poor immune surveillance baked into the cake of CLL patients. When DLBCL shows up as a second cancer, a new clone that does not originate from the original CLL clone, it is called “de-novo” DLBCL (just a fancy way of saying it is a brand new cancer)
The other way in which DLBCL can happen is if your CLL cells pick up additional chromosomal abnormalities and morph into these large diffuse B-cells typical of DLBCL. When this happens the newly formed DLBCL cells share a lot of genetic features in common with the original CLL clone and this can be identified in the lab. Majority of DLBCL cases are of this variety, where the DLBCL cells are transformed CLL cells – hence the name Richter’s transformation.
Hematol Oncol. 2009 Mar;27(1):1-10.
Richter syndrome: molecular insights and clinical perspectives.
Rossi D, Gaidano G.
Division of Hematology, Department of Clinical and Experimental Medicine and BRMA, Amedeo Avogadro University of Eastern Piedmont and Azienda Ospedaliero-Universitaria Maggiore della Carità, Novara, Italy. rossidav@med.unipmn.it
Richter syndrome (RS) represents the clinico-pathologic transformation of chronic lymphocytic leukaemia (CLL) to an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). The clinical definition of RS is heterogeneous, and encompasses at least two biologically different conditions: (i) CLL transformation to a clonally related DLBCL, that accounts for the majority of cases; (ii) development of a DLBCL unrelated to the CLL clone. In clonally related RS, the pathogenetic link between the CLL and the DLBCL phases is substantiated by the acquisition of novel molecular lesions at the time of clinico-pathologic transformation. RS is not a rare event in the natural history of CLL, since the cumulative incidence of RS at 10 years exceeds 10%. Prompt recognition of RS is known to be clinically useful, and may be favoured by close monitoring of CLL patients harbouring clinical and/or biological risk factors of RS development. Conventional risk factors that are independent predictors of RS development at the time of CLL diagnosis include: (i) expression of CD38; (ii) absence of del13q14 and (iii) lymph node size > or =3 cm.Other risk factors of RS development include CD38 genotype and usage of specific immunoglobulin variable genes. The molecular pathogenesis of RS has been elucidated to a certain extent. Acquisition of TP53 mutations and/or 17p13 deletion is a frequent molecular event in RS, as it is in other types of transformation from indolent to aggressive B-cell malignancies. Additional molecular alterations are being revealed by genome wide studies. Once that transformation has occurred, RS prognosis may be predicted by the RS score, based on performance status, LDH, platelet count, tumour size and number of prior therapies.Depending on patient’s age and RS score, the therapeutic options for RS may range from conventional immunochemotherapy to allogeneic bone marrow transplantation.
Copyright 2009 John Wiley & Sons, Ltd.
PMID: 19206112
Who is more at risk?
Earlier articles put the risk of developing Richter’s syndrome at 2-3 % of CLL patients. However, based solely on my anecdotal database of patients writing to me, this has seemed low to me. Now the paper by Rossi et al below confirms my suspicion. They put the risk of Richter’s syndrome at a whopping 10% for patients who have had CLL for more than a few years. The authors identified the following risk factors:
- Elevated CD38
- Absence of 13q14 deletion, the “favorable” FISH abnormality
- Lymph nodes larger than 3 cm
- 17p53 deletion or mutation. This is important. We discussed in a previous article “How to make a good roach killer” that even if FISH report says there is no 17p53 deletion, it is entirely possible to have mutations or epigenetic silencing of the 17p53 gene, preventing it from making proper TP53 protein. The goose is cooked either way.
- High white blood cell counts (WBC), swollen spleen or even percentage or type of bone marrow involvement (diffuse or nodular) did not increase risk of Richter’s transformation, though these factors obviously point to increasing Rai stage of the CLL.
- The authors point out: “The extent of disease has been shown to influence the outcome of RS patients. Consequently, prompt recognition of RS may be clinically useful, and may be favoured by close monitoring of CLL patients harbouring clinical and/or biological risk factors of RS development.”
The link between increased risk of RS and high CD38 expression is interesting. It has been known for a while that CLL cells tucked away in lymph nodes express higher levels of CD38 than CLL cells found in open blood circulation or even the bone marrow. In other words, the CD38 level your lab reported on the basis of a blood test is likely to be an underestimation of the level of CD38 expressed by your CLL cells residing in swollen lymph nodes. Most experts agree that CLL which likes to sit around in lymphoid tissues (“bulky disease”) is more likely to be associated with lymphoma-like tumor, and therefore more likely to increase risk of RS.
The second abstract below from M. D. Anderson highlights another potential risk factor, namely Epstein-Barr virus (EBV). The same group also reported that patients who have had clinically diagnosed mononucleosis in their youth are more likely to have reactivation of EBV infection. As we have pointed out in several articles, once a person is infected with EBV virus, traces of the virus linger in the body for the rest of the life of the patient. Vast majority of our population are carriers of this virus. It seems patients who had full blown mono as kids are more likely to have reactivation of this virus and this abstract suggests it might increase risk of Richter’s transformation as well. We discussed this and several other aspects of EBV ion www.clltopics.net in an article titled “EBV: the enemy within” and I strongly urge you to read it.
Med Oncol. 2007;24(1):17-32.
Recent advances in the diagnosis and therapy of Richter’s syndrome.
Swords R, Bruzzi J, Giles F.
Department of Haematology, University College Hospital Galway, Galway, Ireland.
Richter’s syndrome (RS) denotes the development of aggressive lymphoma that arises in patients with chronic lymphocytic leukemia (CLL). Presenting features typically include a rapid clinical deterioration with fever in the absence of infection, progressive lymph node enlargement, and an elevation in serum LDH. Diagnostic biopsy of affected sites usually reveals large cell lymphomas; however, Hodgkin variant cases have been described. Richter’s transformation occurs in approx 5% of CLL patients and may be associated with infection with Epstein-Barr virus (EBV).Chromosome 11 and 14 abnormalities have also been described as well as tumor suppressor gene defects involving p53, p21, and p27. Treatment options for these patients are limited and includecombination chemotherapy with or without the addition of monoclonalantibodies and stem cell transplantation. Response to therapy is variable and generally short-lived. Median survival is usually in the order of 5-8 mo. More effective management for RS is needed as well as prognostic models that will identify CLL patients at risk of transformation. This review will address the current status of RS and deal with the pathophysiology, diagnostic approach, and treatment of this challenging disease.
PMID: 17673808
“RS Score”
The Rossi article points out that while the prognosis for RS patients is generally considered to be grim, not every RS patient has the same unfavorable prognosis. In their cohort of 148 patients with RS survival ranged from just a few weeks to 15 years! The authors came up with what they call the “RS SCORE” to try and predict who may have a relatively easy time of it and who may not. Patients get one point (demerit) for each of the following:
- Elevated LDH (lactate dehydrogenase)
- Platelet count below 100K
- Reduced physical performance
- Lymph nodes larger than 5 cm
- More than two prior therapies
Patients are assigned to low risk (0-1 demerit points), high to intermediate risk (2-3 points) and high risk (4-5 points).
Please understand their RS score does not predict the likelihood of developing Richter’s syndrome, it tries to predict patient’schances of survival after RS has been diagnosed. Fine need aspiration of the actual contents of the lymph nodes and examination of the sample by a competent pathologist is the most useful method for accurate diagnosis of Richter’s syndrome.
Therapy options
Many of the therapy regimens used to treat RS have their origins in treating aggressive lymphomas. Since the advent of Rituxan, many of these protocols have been changed to add our favorite monoclonal to the line up of other chemotherapy drugs. At present, some of the combinations used to treat RS are
- R-CHOP (Rituxan, cyclophosphamide, vincristine, doxorubicin, prednisone)
- R-hyper CVAD (Rituxan, cyclophosphamide, vincristine, doxorubicin, dexamethasone)
- OFAR(oxaliplatin, fludarabine, cytarabine and Rituxan)
OFAR is the latest and greatest, but even with this combination the median duration of response is only 10 months and 6 month survival rate is a disappointing 60%. The good news is that younger and fitter patients can hope to go through a consolidation therapy with one of these combinations and follow it up with a mini-allo transplant. The estimated cumulative survival at 3 years has been reported to be 75% for patients who received allogeneic SCT after getting a CR or at least PR from the prior consolidation therapy, while patients who responded to consolidation therapy but did not go on to get a SCT the 3 year survival was a bleak 27%. Moral of the story, if you are unfortunate enough to be one of the RS cases, get thee to heavy duty consolidation therapy right away and back it up with a mini-allo transplant!
For patients who are not good candidates for mini-allo transplant, the authors suggest backing up consolidation therapy options listed above with newer drugs such as Bexxar, Zevalin (both of these are variants of Rituxan, anti-CD2o monoclonals, with the big difference that they carry a radioactive payload), Humax-CD20, Revlimid and flavopiridol.
Editorial
There is still a lot that we do not understand about Richter’s syndrome. Giles et al point out that the rate at which CLL patients transform to this much more deadly aggressive lymphoma has been increasing over time. Just in the last decade or so, the incidence has increased from 1-2% to 10-12%. This opens up a can of worms, does the increased rate of Richter’s transformation have anything to do with use of more aggressive chemotherapy drugs? Our favorite villain fludarabine is always good for another black mark in my book.
Giles et al discuss the possible role of EBV viral infection in Richter’s transformation and I suggest you read their actual article to get all the details. While the role of EBV in RS is still an open question, this nasty virus has clearly been identified with the development of other lymphoid cancers such as Burkitt’s lymphoma and Hodgkin’s lymphoma.
While as many as 90% of our population have been exposed to EBV at some point in their lives, the percentage of that come down with full blown and clinically diagnosed mononucleosis is quite small. A while back we did a straw poll on CLL Topics, asking our members to tell us if they have had mononucleosis as kids. A surprisingly large percentage of our people have had full blown mono. This is a potential lead that I hope will be followed up some day by researchers.
You and I can talk about this and other potential linkages until we are blue in the face, nothing will get done and we will get no respect. My goal is to see if I can get a credible research outfit to work with our patient community by following up on leads such as this. Your part of the bargain is that if and when I succeed, each and every one of you promises to spend the 15-20 minutes to participate in an on-line survey. Agreed? Who is going to help us if we don’t help our selves?
41 comments on "Richter’s Syndrome"
Agreed! Thanks so much for this article and all that you do for us, Chaya. I hope you are doing well.
Thanks much for the alert. I’ll be questioning my oncologist if he’s up to date on this.
Del
I’m game too Chaya. Anytime you need my info in a survey, you’ve got it.
Thanks for all you do. There is no way I’d be able to get through all these medical papers.. I get lost in the terminology. Your summaries are clear to me. Thank you for being our ‘translator’! And so much more.
Fangfarrier
A very welcome article, Chaya, bringing us up to date on this important subject, which we should all take note of.
Chaya, count me in on the survey if you can get someone to do it. I had what the doctor called “a slight case” of mono at the age of 18. It definitely would be interesting to see some research done on it.
Of course I am willing to do any survey you think appropriate and useful, if it takes 15 minutes or an hour and 15 minutes.
So glad we have you to look out for us and keep us up to date on new developments. The least we can do is play our part.
Thank you
Agreed for certain. I think a change from 2-3% to 10-12% in a matter of a decade or so is alarming. I reiterate what others have tapped in these comments, a very humble thank you Chaya for the generous sharing of your knowledge and understanding. I, too, will speak to my doctor to ask if he is up-to-date on this topic.
Chaya,
Very interesting. How can I participate in the online survey? (I had full-blown monon early in college…and was diagnosed with CLL in 2003.)
Thanks so much.
I never had mono, but if there are any other surveys you have I’d be glad to do them!
I’ll be glad to do your survey, Chaya. I did have mono when I was 14…it was verified by a blood test.
Thank you for all that you do.
Betsy
I have been a faithful follower of CLL topics since I was diagnosed with CLL in late 2006. Having unfavorable diagnostic markers and an fast increasing WBC I was monitored and followed closely by my local oncologist. I also immediately sought a second opinion at a national cancer center. I was started on FCR in May, 2007. After two cycles of FCR my lymph nodes had not reduced in size and my WBC had not decreased as much as expected. We had a lymph node biopsy done in July, 2007, and analyzed by both the local and major cancer center pathologists. It showed Richter’s Transformation. In August, 2007, I was started on R-CHOP immediately and visited my doctor at the major cancer center. Without equivocation it was recommended I have a SCT. I had a mini-SCT in November, 2007. From initial CLL diagnosis (WBC =16,000), to Richter’s transformation, to actual SCT was only 12 months! So the lesson is, if you have unfavorable diagnostic markers monitor your disease closely and, if initial chemo is unsuccessful and Richter’s appears, consider a SCT ASAP. How am I doing? At 16 months post-transplant everything is fine, all tests are negative for CLL or lymphoma.
Kbpaone:
There is no survey to do – yet. I am going to try to get one the expert centers to do such a survey and if I succeed in doing that then I hope each and every one of you will participate. You can count on it that when (if!) the survey is ready I will give it as much publicity as I can!
Patrick, we would want people who have never had mono to participate as well! How else will we know if the percentage of CLL patients with prior mono is highter than the incidence of mono in the general non-CLL population?
Spike:
Congratulations! I could not be happier for you.
Thanks so much for posting your experience. It underscores the lesson I am trying to drive home here. Richter’s transformation is bloody dangerous, no question about it. BUT it is not an automatic kiss of death. Diagnosed promptly and treated appropriately (including a mini-allo stem cell transplant) it is possible to dodge even this bullet! The problem arises when bad luck is compounded by ignorance on the part of the patient (or local oncologist!) and that is the whole point of my writing this article.
As more than one expert told me, very bulky disease (especially abdominal lymphadenopathy) is a CLL on the verge of becoming a lymphoma – and there are a few hints in literature now that prior history of mono makes that risk just a tad higher.
When the nodes get so very large, many experts start talking of R-CHOP as the therapy of choice and not our more familiar cocktails of FCR or FR. It seems at some point indolent “leukemia” with bulky disease starts acting more like an aggressive “lymphoma”, even if it is not full blown Richter’s transformation. In such cases I can see why standard lymphoma protocols (R-CHOP) may be more appropriate than “kinder” combinations such as FCR.
All the best Spike. I hope you will be our good luck mascot for all patients who have to face this monster.
Chaya,
These are all excellent ideas. My sense from the literature is that the increasing incidence of Richter’s transformation in part reflects physicians and patients collectively being more “tuned in”, hence picking up both the diagnosis of CLL earlier as well as that of DLBCL. Treatment of the CLL may also play a role as you hypothesized.
The salient point (emphasized by Spike’s personal account) is that aggressive treatment of DLBCL may be the most effective approach for longterm survival. It is unfortunate, but those who undergo multiple courses of therapy (effectively in salvage mode) and then undergo transformation are “up against the wall” and much less likely to have a good longterm result.
Another interesting consideration about EBV is that the timing of it’s presence in the body may determine the course of a given lymphoproliferative disorder. PTLD (effectively post transplant lymphoma), for example, has a better outcome in patients who were EBV seronegative prior to transplant (and acquired the EBV from their donor) than it does in patients who had EBV prior to transplant.
Recently some researchers from Japan described a group of older patients with EBV associated with a large B cell lymphomas that superficially resembled classic Hodgkins Disease, but whose course was more aggressive than was that of classic Hodgkins Disease, even when the latter patients were seropositive for EBV, (sorry if this is confusing). I will review the paper, but as I recall the tumors were histologically and behaviorally different, even though both sets of patients had been exposed to the EB virus.
I agree that someone more knowledgable in EBV epidemiology could be very helpful in devising a useful survey, though it would be nice if we could somehow arrange to “donate” serologic blood tests if they are needed. It wouldn’t be as “clean” as having bloodwork all done by one lab, but may make such a study more affordable and hence more attractive to a researcher.
For anyone who might wish to read the article from Japan (which I so poorly described), here is the citation:
Blood, 19 March 2009, Vol. 113, No. 12, pp. 2629-2636.
You can count on me whenever the survey is ready.
Irv Noble
Canada
Chaya,
I’ll participate in a survey whenever you are ready.
I have not had mono.
Take care
Rita
I will certainly participate in the survey when appropriate Chaya.
David
Chaya,
I will be glad to participate in any survey. Please keep us posted! A huge THANK YOU for all that you’ve done for the CLL community. Your ability to condense and summarize all the scientific jargon is very much appreciated. My local oncologist says that I would be a good candidate for a shingles shot but I’m hesitant. Stanford Univ. diagnosed me with CLL in July 2007 and I have never received treatment. Any suggestions or advice? Thanks!
A survey is a great idea. Thanks for b
Chaya, of course count me in. I had documented EBV, mono as we used to call it, back in 1965, and sub acute heptatitis. a bad case they said, so yes, I would take part in any and all surveys,tests etc. and again, thanks for the update and all that you do, beth
Dear Chaya
Im a 50 year old female that was diagnosed with CLL last year.Indolent, however I have now developed severe Rhuematoid Arthritis and diabetes as well. All auto-immune. It seems CLL goes hand in hand with all kinds of other disorders. I had mono about 10 years ago too. I keep waiting for another shoe to drop. You’re a God send to all CLL patients, and I think most of us would do about anything to help you in any way we could.
Thank You for everything
Linda
I have just been diagnosed with CLL in January at 56. I had a diagnosed case of mono when I was 20. I will participate in any survey you do. This website has been the only encouragement I have found since my diagnosis. Thank you so much for all you do.
Cynthia
jt123:
I would suggest that you avoid the herpes zoster vaccine as it utilizes a live virus. Most experts feel that it is not advisable to utilize that type of vaccine in immunocompromised patients such as those with CLL.
Dr. Hamblin did post on this some months ago as did Dr. Koffman. You would probably benefit by reading what they had to say on those blogs and then discussing the matter with your physician.
Good luck,
Rick
Coming from the UK, I’d never heard of mononucleosis. A quick search on the internet revealed it is what we call glandular fever. Hope this helps other (similarly ignorant) UK readers!
D
jt123:
I second Rick’s comments on the shingles vaccine. There does seem to be some controversy on the subject though. The usual guidance for CLL patients is not to use any vaccine that has live virus in it. It is thought that in our immune compromised state the live virus may precipitate the very illness it is supposed to prevent!
However, that usual guidance has been set aside by health officials in the case of the new shingles vaccine. Dr. Hamblin does an excellent job of presenting both sides of the argument on his blog. I suggest you read his comments before deciding. Me, I think prudence is the better part of valor.
Doff, sorry about not mentioning mononucleosis is called glandular fever. That is what it is called in India as well.
Bob Armstrong
I’m in…my original FISH 3 years ago suggested B cell involvement and I have the 17p53 culprit in my basket, too…so far symptom free…
Chaya:
Thank you for your continued support and valuable information-I would participate in any survey that may assist us all in fighting this disease.
Brian
I was mis-diagnosed with tranforming my CLL to Richter’s one year ago. My biopsi was perfomed locally and lab tested locally. The reply from the lab was DLBCL “indicative of” transformation from my CLL.
I went to Stanford for a consultation and was sent to the BMT division. They recommended me for an ALLO and agreed with my local onc of R-CHOP which I completed 6 rounds and then 2 round of rituxan only. As the time neared for my BMT my onc at Stanford asked his lab to perform the same test as initially to verify the transformation. They found it WAS NOT a tranformation, just my good luck to have another cancer. It was “assumed” by all doctors that it was a transformation. Make sure to have any test indicating transformation done more than once.
I am currently 13 months post diagnosis and so far so good. I was able to get a complete remission of the lymphomma and it knocked my CLL down to “normal” ranges.
Just another time that transformation was not a transformation. Just another cancer.
Dear Chaya,
Ditto with me, had mono as a seventeen-year old. Also have had psoriatic arthritis for the last 26 years. I was on Enbrel (TNF Blocker) until my diagnosis of SLL/CLL about a year ago. So far, still watch and wait with good prognostic indicators. Anything I can do to help, I will participate in.
Sam
Thanks 11qRick and Chaya for your advice. I’ll read Dr. Hamblin’s and Dr. Koffman’s blogs. It’s nice to know someone out there “has my back.” Sometimes it feels patients are given health suggestions rather casually. Docs are overwhelmed with their workloads I’m sure.
Gratefully,
jt123
I am a 74 yr. old, physically active female diagnosed with CLL in 1996, treated with FR in 2005, participated in a Revlamid Protocol at NIH in 2007, Campath & R for a few months here at NW in Chicago in 2008,diagnosed Richter’s Syndrome last week & began treatment today with Bendamustine & R. This is the first time my brilliant and famous Dr. Steven Rosen has tried this treatment with Richter’s, and thought you may be interested in my progress. I’m not too computer-savvy, but will be happey to provide information if you would find it useful. Let me know. Jean Paulus
Jean:
I am so sorry you have to face Richter’s. But you sound like the kind of lady that has both the guts and determination to defeat odds that others may find insurmountable! Good luck!
Yes, I would most definitely be interested in learning about how you fare with this novel regimen. You can either post your comments on this website or you can send me your comments as a personal email and I will update the rest of our members. Whatever works for you.
Thank you for offering to share your experience. We are all stronger when we try to help each other.
Best wishes,
Chaya
15 minutes you say. that’s a drop in the bucket comparded to all you do for us! I’ll be more than happy to do a survey. No Mono in the past. But something interesting, I had an emergency spleenectomy last fall due to a fall I took, I was in stage 3 w/ my CLL. when I went back for a check up w/ my onocologist he pronounced me back to stage 0. My brother in law’s friend does research on CLL & advocates removing those pesky organs once they start enlarging.
I have never been diagnosed with mononucleosis.
I’ve read that a rule of thumb is 1% incidence of Richter’s every year, so after 10 years, that would lead to 10%.
I told a famous doctor from MD Anderson that I wanted to avoid FCR because of the risk of Richter’s. He said that I was already at high risk because of my impaired immune system coincidental to having CLL.
I have not had mono, but would do any survey Chaya suggests if it would help.
It seems as if most who post here have great doctors looking out for them and their CLL? Do they discuss your blood work and the various things that are low/high and why? Or other details of CLL?
So many things that CLLers need to look out for, should know, has NOT come from my doctors. It usually just the W&W. I ask about my CLL but they oddly prescribe, CT scans, or many other tests, which come back negative.
I inquired about the radiation, and am told that it’s such a low dose or that it is less than I’d get from the sun in a normal day.
Then for 8+ months they forgot to tell me my igG was low, then a couple weeks after last CBC a nurse calls to tell me he ordered an infusion now, but forgot to tell me. Really? Is this normal?
Another area of complete confusion is the different normal range averages for blood work. The igG varying 200 between the two charts. Is this also normal? Isn’t there a set range to judge from so a patient knows what is a true normal range, and what is low or high.
Thank you Chaya for all the valuable information. I was able to get a second pneumo-vaccine several weeks ago because of your valuable information.
Enjoy your holiday.
I am a 61 one year old female. Last April I had a biopsy done on my right axilla done at my local hospital. Diagnosis was CLL. Saw a local dr. who suggested treatment with treanda. Decided on a second opinion and saw dr. at Dana Farber. Was uncomfortable with the interview. Without much conversation, he suggested trial of revlimid. A few weeks later, when I called to accept, he said by numbers weren’t high enough and to call back when they were. Meanwhile, decided to see another doctor in NYC who said he wanted to start from scratch and ordered another biopsy, on the left side. This time pathology said it was a Richter’s transformation. I will be starting next with CHOP-R. Fortunately, my LDH levels are low and I have not any treatment yet. I do have a 17p deletion though. Here’s hoping for the bestbut I don’t know what the statistic are. Just glad I got more information.
Thank you again Chaya,
Your undaunted research and commitment to sharing such is welcomed and insightful to all of us.
I have been very active in my therapy for Richter’s which was diagnosed 39 months ago, the docs say I have a very unusual case as LDH, beta 2 microglobulin are normal as are other favorable tests and 17p has no deletion. Yet three months ago three lymph nodes had swollen and were given a FNB to again diagnose RS.
As was revealed to me by the PET scan technician tumors are “hungry for glucose” and that is the reason that one fasts to lower the blood sugar before getting the radiolabled glucose infusion for the PET. As you know the suspected tumors, since they have been deprived of sugar due to the fasts will “suck up” the radiolabled glucose and “light up” the PET photos which indicate that the area lit up may be cancerous.
Reasoning with my onc he agreed to give FCR using Dextrose 5% (a form of sugar) and not the standard saline vehicle after I fasted. The lymph nodes would attract the FCR due to the above more so then the saline was the reasoning.
Upon the first treatment that night I felt that the swelling may have been effected, on the second night there was a definite shrinking of the nodes and on the third night the swelling disappeared. A follow up PET showed no cancerous tissue anywhere in the body. My docs statement on this was, “Remarkable!”
Also I took your research to heart (Let them eat yogurt) and have been making the Lactobacillus Ramosus GG or LGG (brand name Culturelle available at Wal Mart and Target) into organic yogurt after my last FCR treatment and found that the uncomfortable feeling in the gut dissipated in 6 hours, in 12 hours it had vanished and in 24 hours regularity returned. Of course I did not take the antibiotics and anti viral meds the doctor prescribed as they would cancel out the effects of the LGG as researched in the paper,
Upon further research I found that a Dr. C.K. Wong at the Prince of Wales Hospital in Hong Kong was giving his breast cancer patients standard chemotherapy and polysaccharides to boost immune function and considering the problem with all of us is a weakened immune system I have opted to use his well documented research and use such polysaccharides as adjunct along with chemo.
What concerns me the most is the prognosis for Richter’s: the doctors are saying there is no cure outside of the risky allogenic transplants. However I would like to qualify that by saying that, “Using the accepted therapies laid out by the Western educated oncologist there is no cure for Richter’s transformation outside of allogenic transplants”.
You have demonstrated in your site that the LGG re-establishes the mucus membrane in the GI, and I for one have found that it has worked yet when I suggested this to my oncologist he did not know about it and advised against it and instead he preferred the traditional treatment of antibiotics and antivirals which have their side effects and are inorganic.
It seems to me that the practice of medicine must embrace the art of medicine which means to that one be open to scrutinized / comparative research by others. What is the norm for the Western educated physicians is the embracing of clinical trials. Keep in mind that thalidomide was approved and resulted in horrendous birth defects as well as a recent approved drug which was withdrawn due to the fact that it caused heart failure. This is not to say that Western medicine has not been effective but as all knowledge is limited and includes clinical trials. Which the doctors which I have interviewed have agreed to.
You say we must be our own advocates and in this I completely agree with the caveat that to be aware of all of the bogus remedies as well as the well documented research and utilize such for our well being is a must to enhance the bodies health and lessen the side effects caused by well meaning oncologists therapies.
Kind regards,
RW
My mom was just diagnosed with richter’s transformation. She will be starting the OFAR protocol next week. If the OFAR can knock it down enough she will then be given the experimental CLL autologous vaccine. Luckily, she lives in Houston, TX and has a wonderful oncologist who presented her case to the tumor board at MD Anderson. I am so worried. She is only 64. I am just looking for some hope out there. Is there anyone who has survived this without having a sct? The doctors have not mentioned anything about doing one. She also has had breast cancer x2 and was treated each time with chemo (which is where they think she got CLL ) starting 15 years ago. Chaya, I have been reading all over your site and I am so sorry for the loss of your husband. You are a strong woman to continue with this.
Thanks for any information
Alison
Jean and Chaya–
I am undergoing a biopsy next week to determine if my CLL has morphed into the more aggressive DLBCL. I was originally diagnosed with SLL/CLL in 2000 and underwent PCR therapy from 2/09 to 6/09. Like Jean, I have also seen Dr. Steve Rosen at Northwestern in Chicago (for a second opinion in 2009). My Iowa oncologist is talking about Tranda/Rituxan therapy if Richter’s syndrome has occurred, and I’m wondering how Jean made out with that treatment in 2009.
Thanks very much!
Judy
Jean and Chaya–
Re the email I just sent, this was in reference to your emails of March 24, 2009, and it’s Jean Paulus.
If anyone else has information on recent treatment for Richter’s transformation, it would be most welcome. Thank you!
Judy
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