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Richter’s Syndrome

What is Richter’s syndrome?

Richter syndrome, also called Richter’s Transformation, is a complication of chronic lymphocytic leukemia (CLL). It occurs when CLL quickly changes into a more aggressive form of large cell lymphoma. The majority of cases see CLL transform into diffuse large B-cell lymphoma (DLBCL).

 It is a very rare complication, occurring in 2-10% of all of those with CLL. 

What are the symptoms of Richter’s syndrome?

The most common symptom of this complication is lymphadenopathy, which is when the lymph nodes expand suddenly. This expansion brings swelling in the neck, axilla, abdomen, and groin. Other symptoms include:

  • Unexplained weight loss
  • Fatigue
  • Night sweats
  • Fever
  • Shortness of breath
  • Dizziness
  • Possible palpitations
  • Low platelets

What causes Richter’s syndrome?

The exact cause of this complication is unknown. There are certain factors that are believed to increase the risk of Richter’s syndrome. There are certain inherited genetic characteristics that increase the likelihood of developing this complication, as well as certain genetic mutations. Features of the cancer cells themselves may also increase the chance of Richter’s syndrome.

How is Richter’s syndrome diagnosed?

An excisional biopsy can be used to diagnose this syndrome, as can a CT scan or ultrasound guided biopsy. 

What are the treatments for Richter’s syndrome?

Treatment for Richter’s syndrome depends on what form of lymphoma CLL develops into. An immuno-chemotherapy regimen called R-CHOP is usually the first form of treatment, but other forms of chemotherapy may also be used. A stem cell transplant is an option as well. There are also clinical trials being conducted of drugs that may soon be an option. 

Where can I find out more about Richter’s syndrome?

Richter's Syndrome Articles