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Genetic and Rare Diseases Information Center (GARD)

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.


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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

  1. Cancer Research UK. Richter's syndrome. Last updated: 05/01/2013; Accessed 1/3/2014.
  2. Tsimberidou AM, Wierda WG, Wen S et al. Phase I-II clinical trial of oxaliplatin, fludarabine, cytarabine, and rituximab therapy in aggressive relapsed/refractory chronic lymphocytic leukemia or Richter syndrome. 2013 Oct; 13(5):568-74. Accessed 1/3/2014.

GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

    Finding Treatment Information

Management Guidelines

  • The American Cancer Society provides information about the treatment of Richter syndrome within a report on chronic lymphocytic leukemia. Click on the link above to access this information.
  • The National Cancer Institute (NCI) provides information related to the management of Richter syndrome. Information about treatment of adult non-hodgkin lymphoma and chronic lymphocytic leukemia can be accessed at the above links.

Clinical Trials & Research for this Disease

  • lists trials that are studying or have studied Richter syndrome. Click on the link to go to to read descriptions of these studies.
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".
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.