Richter’s syndrome is the rapid transformation of chronic lymphocytic leukemia (CLL) into a more aggressive form of cancer associated with a less favorable outlook.
Richter’s syndrome is a rare complication of chronic lymphocytic leukemia (CLL), a type of cancer that affects your bone marrow and blood. It occurs when CLL transforms into a more aggressive form of lymphoma.
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Richter’s syndrome is commonly referred to as Richter’s transformation. According to Cancer Research UK, the condition affects up to 1 in 10 people with CLL.
Keep reading to learn more about the possible causes, symptoms, and treatments for Richter’s syndrome.
The exact cause of Richter’s syndrome is unknown.
However, researchers have identified several risk factors that may increase the chances that CLL will change to Richter’s syndrome. These may include:
- being 65 years old or older
- being assigned male at birth
- having genetic or biological changes or mutations, such as TP53 mutation status or unmutated IGHV status
- having biomechanical differences, such as abnormal platelet counts or immunoglobulin counts
- having had stem cell transplant, radiation therapy, or chemoimmunotherapy
If Richter’s transformation of CLL occurs, you may experience symptoms known as B symptoms. These
- fever
- drenching night sweats
- rapid unexplained weight loss
Another sign of Richter’s syndrome is swelling of your lymph nodes. You may notice swelling in your neck, groin, abdomen, or other areas with lymph nodes.
If the cancer has spread outside your lymph nodes or you develop anemia, you may also experience the following symptoms:
- feeling full soon after starting to eat
- skin rashes
- headaches
- blurred vision
- shortness of breath
- restless legs
- fatigue
- lethargy
- chest pain
- reduced exercise tolerance
- weakness
Blood tests
If you have CLL and experience any symptoms, speak with a member of your treatment team as soon as possible. They will likely want to run tests to find out whether Richter’s transformation has occurred.
They may start with a lymph node biopsy to see if CLL has transformed into a more aggressive lymphoma. They may also order other tests, such as:
- blood tests
- bone marrow biopsy
- PET scan
Richter’s syndrome typically does not occur until
Currently, the most common initial treatment for Richter’s transformation of CLL is chemoimmunotherapy.
This may involve taking a combination of steroids and immunotherapy drugs. According to Cancer Research UK, a common combination is called R-CHOP, which includes the drugs:
- rituximab
- cyclophosphamide
- vincristine
- prednisolone
After the initial treatment, your doctor may recommend secondary chemotherapy regimens.
They may also recommend a stem cell transplant if your initial treatment helps you attain remission. A transplant is when a doctor transfers healthy stem cells from the blood or bone marrow of a donor to replace damaged or dead stem cells in your body.
Researchers have begun several clinical trials of advanced treatment options to help improve the overall outlook of Richter’s transformation.
Current trials are looking into the safety and effectiveness of:
- immune checkpoint blockers
- Bruton tyrosine kinase (BTK) inhibitors
- venetoclax
- combinations of bispecific antibodies
- chimeric antigen receptor T cells
The life expectancy of someone with Richter’s transformation of CLL is low. With currently available treatments, the average life expectancy is 6 to 12 months after diagnosis.
Targeted therapies also don’t appear to have much impact.
For instance, a 2017 study involving 71 people with Richter’s syndrome found that the average survival time of people who received novel treatments like BTK inhibitors or venetoclax was 3.3 months.
One factor affecting the outlook is clonality, which is when two tumors come from the same cell. About 8 in 10 cases of Richter’s syndrome is clonally related to the initial CLL.
The median survival rate for clonally related Richter’s syndrome is 14 months, while the median survival rate for non-clonally related Richter’s syndrome is about 5 years.
Other known independent factors that decrease life expectancy in Richter’s transformation are:
- LDH levels greater than 1.5 times the upper limit of normal
- low platelet counts
- tumor size of 5 centimeters or larger
- more than one previous therapy
A Zubrod scale performance status greater than 1 may also decrease life expectancy. This is a scale of 0 to 4 that doctors
If CLL transforms into Hodgkin’s lymphoma, the outlook improves, with an overall survival rate of about 13 years.
Is Richter’s syndrome terminal?
Yes, in most cases, research suggests that the average life expectancy for someone with Richter’s syndrome is 6 to 12 months after diagnosis.
However, it’s important to note that your life expectancy may vary greatly depending on various factors.
Is Richter’s transformation curable?
There’s currently no cure for Richter’s transformation, but research suggests treatment could help up to 1 in 5 people achieve remission.
Richter’s syndrome is an uncommon condition in which CLL transitions rapidly to a more aggressive form of cancer. It is often diagnosed 2 to 4 years after the initial CLL diagnosis, but it can happen at any time.
Symptoms typically include night sweats, fever, and rapid, unexplained weight loss. Doctors will likely conduct blood tests to check for this change.
Current treatments are relatively ineffective, but future clinical studies may find safe, effective treatment options.