Richter's in a Nut Shell

Richter's in a Nut Shell

'Richter's transformation, or Richter's syndrome, is an uncommon clinicopathological condition observed in about 5% to 10% of patients with chronic lymphocytic leukemia (CLL). "Richter's transformation" refers to the development of aggressive lymphoma during the course of CLL. Diffuse large B-cell lymphoma (DLBCL) occurs in the majority of cases of Richter's transformation.

Clinically, patients with Richter's transformation present with an aggressive disease course with rapidly enlarging lymph nodes, hepatosplenomegaly, and elevated serum lactate dehydrogenase levels (LDH). Specific risk factors for the development of Richter's transformation in a patient with CLL have yet to be identified; however, TP53 disruption, c-MYC abnormalities, unmutated immunoglobulin heavy chain (IGHV) < 2%, non-del13q cytogenetics, CD38 gene polymorphisms, stereotypy, and VH4-39 gene usage may predispose to Richter's transformation.

The prognosis is generally poor, with a median survival of about 10 months.

Development of rituximab (Rituxan)-containing intensive chemotherapy regimens and chemo-immunotherapy regimens (eg, R-HyperCVAD [rituximab plus hyperfractionated cyclophosphamide, doxorubicin, vincristine, and dexamethasone] or OFAR [oxaliplatin (Eloxatin), fludarabine, and ara-C]) have improved response rates but have not clearly affected long-term outcomes.

Allogeneic stem-cell transplantation may offer a chance for prolonged survival.'

Article

cancernetwork.com/print/art...

Dr. Peter Hillmen from Leeds on Richter's

cancernetwork.com/print/art...

Dr. Jeff Sharman on Richter's

cll-nhl.com/2013/01/richter...

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  • Nice summary from one who knows it too well. NOTCH1 may be the most important risk factor.

  • I have just read the above articles about Richter's transformation. Not easy reading - not cheerful reading. They say it is an "uncommon... condition" but then go on to say it is "observed in 5% to 10% of CLL patients", which doesn't sound so uncommon to me. Quite a few of us on this site are likely to go that way.

    I'm aware that you, Clcanada, have got this "RT" condition yourself. Looking back at other posts of yours, I also see that you're still suffering from painful shingles, as well as facing a very poor prognosis, humanly speaking. That must be very, very hard. Most of us who visit this site are in earlier stages of CLL and we live in hope... But there will come a time for many of us, when we have to accept that our time on this earth is now quite limited. Which is something we maybe all need to come to terms with. There is a balance to be kept, between continuing to hope for the best, yet being prepared for other options. I'm not sure if that is something people would want to discuss here, or not...

    To end on a more positive note, I want to say that I really value all your contributions to this site, Cllcanada. I am grateful that you put so much time into responding to people's queries, in a lot of thoughtful, well-informed detail.

    Thank you,

    PaulaS

  • Hi CLL Canada,

    Thanks for this very straightforward and realistic account of Richters - I was just looking for a succinct way of summarizing it for my research. I've been looking at several HRQOL studies (that's health related quality of life to you and me) for CLL patients. You won't be surprised to find that there is a dearth of research of this nature around CLL, but the studies I have found all point to living with the fear of further complications from the disease as a factor impacting on quality of life in our disease group (although they do little to explore those in any further detail, and I'm hoping my work with online communities might help to fill that gap).

    I'm looking at work around perceptions of health-related risk in the general population and relating it to people living with chronic cancers such as CLL. I think too little attention is given to the difficulties of adjusting to living with a body which we have already come to see as 'dangerous' or threatening in its own right. The constant awareness of secondary cancers and Richter's, not to mention opportunistic infections means our perception of being at risk are significantly higher than that of the general population, and indeed higher than those of people surviving with many other forms of cancer for whom the hope of 'cure' is more realistic.

    I agree with Paula that this means we need to be realistic about the possibilities of our disease course, whilst not abandoning the all-important element of hope and the ability to enjoy our lives in the moment. I think it's interesting how what we hope for changes all the time as our disease progresses and we develop new benchmarks for our aspirations. These are fundamentally important issues for all of us, and no-one should feel anxious about discussing them here

    Like Paula, I really appreciate your well-informed, honest, and deeply human presence, not just here but in all of your other online guises around CLL. I think about you, and your current situation a lot, and I wish you all the very best as you face up to a challenge that any one of us here may need to address in the future.

    I guess you could say I'm a fan of yours CLL Canada, and I, like many others here I'm certain, am rooting for you :-)

    Jules

  • Although this transformation may only be 5-10% I knew of a lady similar age as myself with CLL who died through Richters. Although you cannot spend every waking hour thinking about CLL you do have to consider it every waking hour. Am I doing too much? Am I hygenic enough? Do I have a good consultant? Am I getting an infection or should I just ignor it? Its just living with a chronic disease but I just want it to go away.

  • Thanks everyone for you comments...

    I think there is an assumption that hope diminishes as the prognosis changes from years to months... well it doesn't. If anything hope increases as time shortens.

    Yesterday, I really hoped I could manage to walk to the end of the block and back, in the glorious Spring sunshine... I did...

    Later, I really hoped I could stand long enough in the kitchen, to slice mushrooms and cook a steak for dinner... I did...

    Small victories...

    Today...I hope to take out my camera and photograph the early rhododendrons... I will...

    My hope is renewed daily.

    I think this is a very important discussion...

    ~chris

  • Chris,

    Your description of hope increasing as time shortens, is very helpful. I agree - our hopes may change as time passes, but they can be equally, if not more, life affirming than our former hopes. I have enormous respect for your attitude in all this.

    I think it's something we can all work towards (your view of hope), though it might take some disciplining of our thoughts, to set our minds to think that way, and not let more negative thoughts overwhelm us.

    Anyway, just reading your words this morning has been an inspiration and encouragement to me.

    Thank you and bless you,

    Paula

  • Chris,

    What's your take on this report?

    Regimen Shows Promise for DLBCL Lymphoma Patients With Cardiac Conditions

    cancernetwork.com/news/regi...

    "Patients with diffuse large B-cell lymphoma (DLBCL) and coexisting cardiac disease represent a treatment challenge, as they are often ineligible to receive anthracycline-containing chemotherapy regimens. A new phase II trial found that a regimen containing rituximab, gemcitabine, cyclophosphamide, vincristine, and prednisolone (R-GCVP) is active and reasonably safe in this patient population."

    Neil

  • This is basically RCHOP with the Doxorubicin switched out for gemcitabine. I would think it would be less cardiotoxic.

    With RCHOP you can only go to the well once, this might offer second or third line treatment to elderly DLBCL patients, which would be great, but this study was first line treatment.

    Not certain how this would effect Richter's since it isn't your standard DLBCL types, but probably a third variant, research is ongoing...

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