Richter transformation is a rare change that can happen in people with chronic lymphocytic leukemia (CLL). Learn the signs, how doctors diagnose and treat it, and what may affect prognosis.
What is Richter transformation?
Richter transformation, also called Richter syndrome, happens when CLL changes into a faster-growing type of lymphoma. About 2% to 10% of people with CLL develop it.
In most cases, CLL changes into diffuse large B-cell lymphoma (DLBCL). This is a fast-growing type of lymphoma. Less often, it changes into Hodgkin lymphoma.
Richter transformation may be clonally related or clonally unrelated to CLL. Clonally related means the lymphoma grew from the same cells as the CLL. Clonally unrelated means it developed from different cells. This difference can affect prognosis.
Who's at risk of Richter transformation?
Richter transformation can happen at different points after a CLL diagnosis. Some people develop it before receiving CLL treatment.
Certain features are linked to a higher risk. These include changes in the TP53 or NOTCH1 genes, unmutated IGHV, and a complex karyotype. A complex karyotype means the CLL cells have several chromosome changes. Larger lymph nodes and more advanced CLL have also been linked to a higher risk.
Having these risk factors does not mean Richter transformation will occur. Experts do not recommend starting CLL treatment early based only on a higher risk of transformation.
Warning signs and symptoms of Richter transformation
A common sign is a lymph node that grows quickly. One area may also grow much faster than other areas.
Other possible signs include fever without a known cause, drenching night sweats, unintended weight loss, and an enlarged spleen. Blood tests may show a fast rise in lactate dehydrogenase (LDH). LDH is an enzyme that can rise when cells are damaged or growing quickly. Anemia, or a low red blood cell count, and low platelet counts may also occur.
These symptoms can have other causes. Tell your care team about new symptoms or sudden changes.
How is Richter transformation diagnosed?
If your doctor suspects Richter transformation, a positron emission tomography-computed tomography (PET-CT) scan may be ordered. This scan can help doctors identify areas that are suspicious for Richter transformation and select the best area to biopsy.
A biopsy removes tissue so it can be studied under a microscope. It is needed to confirm Richter transformation. Doctors prefer to remove a lymph node when possible. If that cannot be done, several core tissue samples may be taken. A fine needle sample alone is not enough.
The tissue may also be tested to find out whether the lymphoma is related to the original CLL cells. This information can help doctors better understand your prognosis.
How is Richter transformation treated?
There is no single treatment plan for Richter transformation. Treatment depends on the type of lymphoma, past CLL treatments, overall health, and other test results.
R-CHOP, a combination chemoimmunotherapy regimen, has commonly been used. However, responses may not last long. Because of this, a clinical trial may be considered.
Researchers are also studying targeted treatments, bispecific antibodies, and chimeric antigen receptor T-cell therapy (CAR-T). CAR-T changes a person's immune cells so they can better recognize cancer cells. Some people who respond to treatment may be considered for an allogeneic stem cell transplant. This type of transplant uses blood-forming stem cells from a donor.
Your care team can explain which options may fit your health and treatment history.
What is the prognosis for someone with Richter transformation?
Prognosis varies. It can be affected by genetic features, past CLL treatment, response to treatment, and whether the lymphoma is related to the original CLL cells.
Historically, the median overall survival for the most common type of Richter transformation has often been about one year. The median is the midpoint. This means that half of the people in a study lived longer, and half lived for less time.
Studies have found longer survival among people with clonally unrelated Richter transformation. Some studies have also found longer survival among people who had not received CLL treatment before transformation.
These numbers describe groups and cannot predict what will happen to one person. Your care team can explain how your health and test results may affect your outlook.
Living with a Richter transformation diagnosis
A Richter transformation diagnosis can bring new tests and treatment choices. Support from your care team can help you understand these changes.
Coping resources: Cancer centers may offer social workers, counselors, support groups, and financial support. Ask your care team which services are available.
Questions to ask your oncologist: Consider asking what type of Richter transformation you have, what your test results mean, and which treatments are available. You can also ask whether a clinical trial may be an option.
Finding a specialist: Because Richter transformation is uncommon, you may want to see a Richter transformation specialist who has experience treating it. A specialist can review your results and discuss treatment options with you and your local oncologist.
What are the symptoms of Richter transformation?
Possible signs include fast-growing lymph nodes, fever, drenching night sweats, unintended weight loss, an enlarged spleen, and changes in blood test results.
What is the treatment for Richter transformation?
Treatment varies based on your health and test results. Options may include chemoimmunotherapy, newer treatments, clinical trials, CAR T-cell therapy, or an allogeneic stem cell transplant.
How is Richter transformation confirmed?
A biopsy is needed to confirm Richter transformation. A PET-CT scan can help doctors choose the best area to biopsy.
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