Understanding Richter's Transformation
- A small subset of people with CLL will develop Richter’s Transformation, which is when the disease transforms into a faster growing lymphoma.
- Doctors are unsure exactly why Richter’s Transformation occurs, but it does impact how the cancer can be treated.
- The disease may be treated with standard B-cell lymphoma treatments, or patients may be encouraged to enroll in clinical trials.
This new cancer can evolve from mutations within CLL cells or from another B-cell, and it can occur both before and after treatment for CLL. About 2 to 9% of CLL patients may experience their disease transform.Read More
Symptoms that may indicate Richter’s Transformation include:
- Feeling more tired than usual
- Running a fever
- Having night sweats
- Losing weight without trying
- Getting short of breath with little exertion
- Feeling dizzy
- Bruising or bleeding more easily than usual
CLL is a slow-growing cancer. For many patients, the disease progresses so slowly that they can live years without symptoms or the need for treatment. However, when Richter’s Transformation occurs, the treatment plan will need to change because the cancer will be more aggressive.
“It usually is a transformation from CLL to diffuse large B-cell lymphoma. This requires different therapy,” Dr. Lamanna explained. In rare instances, a CLL will transform into a Hodgkin’s lymphoma.
Dr. Nicole Lamanna explains what Richter’s Transformation is and how it is treated.
When the most common type of Richter’s Transformation occurs, and a CLL turns into a diffuse large B-cell lymphoma, there are a few treatment options:
- Enrolling in a clinical trial
- Rituximab-based chemoimmunotherapy
- If chemoimmunotherarpy works, an allogeneic transplant
If chemoimmunotherapy does not work and a patient doesn’t wish to, or cannot, enroll in a clinical trial, another option is to treat patients with immunotherapy drugs nivolumab (brand name: Opdivo) or pembrolizumab (Keytruda). The immunotherapy drugs may be given with ibrutinib, which is a BTK-inhibitor (meaning it targets abnormal cells).
Dr. Lamanna stressed that CLL that has transformed becomes much more difficult to treat, but there are many investigations underway looking into the best ways to control it. “This is an area under a lot of investigation,” she said. “We’re looking for new therapies all the time.”
How is Large B-Cell Non-Hodgkin Lymphoma Treated?
The treatment route for large B-cell lymphoma depends on several factors, but if you experience Richter’s Transformation, it’s important to be aware of what some of the options are so you can make an informed treatment decision with your doctor.
The disease is sometimes treated with a cocktail of chemotherapy drugs such as R-CHOP — the monoclonal antibody rituximab (Rituxan), plus a combination of three chemotherapy drugs and the steroid, prednisone. Another treatment option includes a different monoclonal antibody called obinutuzumab (Gazyva) plus other combinations of chemo drugs such as EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, hydroxydaunorubicin) or ICE (ifosfamide, carboplatin, etoposide).
Dr. Adrienne Philips, a medical oncologist at Weil Cornell Medicine, told SurvivorNet that when it can be used, the R-CHOP combination can be very effective.
Dr. Adrienne Philips explains how the R-CHOP combination works for B-cell lymphoma.
“R-CHOP has been a standard regimen treatment for aggressive non-Hodgkin lymphomas of the B-cell subtype for many years,” Dr. Philips said.
“The drugs in R-CHOP work in combination. It’s hard to isolate the function of any one medication because they haven’t been studied as single agents in B-cell lymphoma … For whatever reason, the lymphoma cells that should have been programmed to die are not dying. So, the R-CHOP combination works together to encourage cells to undergo what’s called apoptosis, or programmed cell death.”
Questions to Ask Your Doctor:
- What are the therapy options after Richter’s Transformation?
- Did my disease develop from CLL cells or other B-cells?
- Is the R-CHOP combination an option I should consider?
- Are there any clinical trials I should consider enrolling in?