What Is Richter's Transformation?

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CLL expert Dr. William Wierda from MD Anderson Cancer Center in Houston describes Richter’s transformation. He discusses how the condition is treated and the prevalence of CLL patients that experience Richter’s transformation.

The Ask the Expert series is sponsored through an educational grant to the Patient Empowerment Network from Pharmacyclics, Inc

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Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you.

Jeff Folloder:

Dr. Wierda, one of our subscribers wants to know what is Richter's transformation?

Dr. Wierda:

So Richter's transformation is if the disease converts to a more aggressive form.  There are other types of B-cell malignancies that are more aggressive than CLL.  CLL is a low-grade or indolent B-cell malignancy.  Other examples of higher grade or aggressive B-cell malignancies or one good example is what's referred to as diffuse large B-cell lymphoma, and those cells under the microscope appear different than CLL cells.  They're larger, they're more active, they're dividing more rapidly, there's not necessarily dying, and those are all features of what's referred to as Richter's transformation where the CLL cells have converted into a more aggressive form.

There is a sub-group of patients who have Richter's transformation who don't have this diffuse large B-cell lymphoma histology but have a Hodgkin's appearance under the microscope.  That's a minority of patients.  It's about 10 to 15 percent.  Most of patients who have Richter's transformation have a diffuse large B-cell histology.  It's a more aggressive appearance under the microscope, and certainly clinically it's a more aggressive disease than CLL.  It grows more rapidly.

It tends to be resistant to standard chemotherapy—it is resistant to standard chemotherapies that we use to treat CLL, and we have to modify the treatment to use more aggressive regimens like we use for lymphoma, like we typically use for diffuse large B cell.  And also if patients have a diagnosis of Richter's transformation, we're working towards getting them to transplant, because we know transplant has been associated with long-term survival in patients who have Richter's transformation, better survival than has been associated with using just standard chemotherapy.

Jeff Folloder:

And how many CLL patients should expect to encounter Richter's transformation?

Dr. Wierda:

It depends on what series that you look at in terms of the literature and what's been reported. In general, it's between 10 and 15 percent of patients over the entire course of the disease.  There are some things that we know put patients at higher risk for Richter's transformation.  One of them is treatment, so it's unusual to see Richter's transformation—it's very unusual to see Richter's transformation in patients who haven't had any treatment or as their first feature when they're diagnosed, but it's much more common in patients who have had prior treatment.

And we're right now working on our database, and we have about 500 patients in our database who have been diagnosed with Richter's transformation, and we're going back and reviewing the features for those patients and doing an analysis.  We're making a concerted effort right now to specifically study Richter's, and it's been a very difficult entity to treat.  Patients with Richter's transformation are difficult to treat, because they're typically resistant to chemotherapy.  We're trying to get them to transplant, and that's been a challenge also.

So we need new treatments for those patients, and we need to better understand the disease or the transformation itself. What are things that cause Richter's transformation?  Are there gene mutations that occur that convert a patient from CLL to Richter's transformation is something that we're evaluating. Having an understanding and appreciation of those things will help us to develop new treatments and potentially prevent it in the future.

Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you.

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Page last updated on March 22, 2016