Great progress has been made in treating chronic lymphocytic leukemia, but sadly the outcomes for Richter’s Transformation (RT)) or Richter’s Syndrome (RS) remain dismal. For more basic information on RT aka RS see: https://cllsociety.org/2017/04/richter-transformation/. For more in general including clinical trials, see: https://cllsociety.org/?s=Richter
Briefly, RT is when the usually slow growing CLL transforms or changes into usually a more aggressive type of lymphoma called Diffuse Large B-cell Lymphoma (DLBCL). There are other less aggressive and less common types of transformation.
I interviewed Dr. Matt Davids of Dana Farber Cancer Institute in Boston, MA at EHA 2019 about his research that he was going to present a few weeks later at another hematology meeting in Lugano, Switzerland.
- A review of 70 CLL patients who developed RT showed they only lived on average between 3-6 months from time of diagnosis.
- A potent aggressive chemo called EPOCH has about a 20% chance of getting patients into a complete remission (CR). We know that getting to CR offers a chance at a cure. EPOCH consists of:
- By adding venetoclax to EPOCH, 2/3 of patients are getting into CR.
- Venetoclax may help chemo to work better and has some activity on its own in RT.
- As RT is so fast growing, the ramp up of venetoclax has been safely sped up to 5 days instead of the usual several weeks, time that many of these patients simply don’t have.
- Once in CR, some patients are going for allogeneic hematopoietic stem cell transplants and most are still alive one year later.
- Infections are a significant risk as is common in all powerful chemo cocktails
Richter’s remains a major unmet need in CLL and the only way we are going to improve outcomes will be with clinical trials. Trials like this that are combining aggressive chemo with a powerful novel agent are offering hope.
Other trials are using combinations with checkpoint inhibitors, also with promising results.
Transplants are usually recommended to “consolidate” therapy, in other words, once the RT is knocked way back, a transplant can turn that remission into a possible cure.
Here is my interview with Dr. Davids: