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Genetic and Rare Diseases Information Center (GARD)

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Richter syndrome


Other Names for this Disease

  • Richter transformation
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My uncle has been diagnosed with Ricter syndrome. His doctor mentioned stem cell transplant as a possible treatment. Can you let me know about any advancements in treatment of this disease which could help him?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

Are there any recent advancements in the treatment of Richter syndrome?

Monoclonal antibodies (MABs) are a type of biological therapy. They are man-made proteins that target specific proteins on cancer cells. MABs are a fairly new treatment for cancer. Doctors often use the MAB drug called rituximab along with  chemotherapy and steroids to treat Richter syndrome. Researchers in a trial called the CHOP-OR study are studying whether a new biological therapy similar to rituximab can make CHOP chemotherapy work better. The new biological therapy drug is called ofatumumab (Arzerra). People who have been recently diagnosed with Richter syndrome can participate in this study. The study has two parts. First, patients have ofatumumab with CHOP chemotherapy to eliminate the lymphoma (this is called induction treatment). They then have more ofatumumab on its own to try to stop the lymphoma from coming back (this is called maintenance treatment). CLICK HERE to learn more about this study.

Stem cell transplant is another way of treating Richter syndrome. While only a few people have undergone stem cell transplant for treatment of this disease, so far it has appeared to work quite well. The disease was controlled for longer than in people having normal dose chemotherapy. However, because stem cell transplants have serious side effects and complications, they are only suitable for a small group of people. More research is needed before we can truly find out how well stem cell treatment works for people with Richter syndrome.[1]

A recent study showed that a chemotherapy regimen called OFAR (a combination of oxaliplatin, fludarabine, cytarabine, and rituximab) had significant antileukemic activity in patients with Richter syndrome and relapsed/refractory CLL. Patients who underwent stem cell therapy as post-remission therapy had even more favorable outcomes.[2]
Last updated: 1/3/2014

References
Other Names for this Disease
  • Richter transformation
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.