How Common is Richter’s Transformation?
Richter’s transformation is not common. It happens in about 2 to 10 out of every 100 people with CLL or SLL.
Why Does It Happen?
Doctors do not know the exact reason why Richter’s transformation happens. It may happen when cancer cells change over time. These changes can help the cells grow faster and act differently from regular CLL or SLL cells.
Some people may have a higher risk. Risk can be linked to certain changes in the cancer cells, such as changes in genes called TP53 or NOTCH1. But having these changes does not mean Richter’s transformation will occur.
It is important to know that Richter’s transformation is not your fault. There is nothing you did to cause it.
Richter’s transformation can come on quickly. Signs may include:
- Lymph nodes that grow fast
- Fever
- Night sweats
- Weight loss
- Feeling very tired
- Pain or fullness in the belly
These symptoms can also happen for other reasons. That is why it is important to tell your doctor about new or fast-changing symptoms.
Doctors may start with a physical exam and blood tests. They may check your lymph nodes, spleen, and liver. They may also look at blood counts and lactate dehydrogenase (LDH) and uric acid levels.
Your doctor may order a PET/CT scan to show areas of the body where the cancer may be more active. A PET/CT scan can help doctors choose the best place to take a biopsy.
A biopsy is the main way to confirm Richter’s transformation. In a biopsy, a doctor removes a small piece of tissue, often from a lymph node. A lab then checks the tissue under a microscope.
Treatment depends on your health, your type of lymphoma, whether your lymphoma evolved from CLL/SLL cells or not, your past CLL or SLL treatments, and your goals. Depending on these factors, treatment may include chemoimmunotherapy, targeted therapy (such as BTK inhibitors, immune checkpoint inhibitors, and bispecific antibodies) stem cell transplant, CAR T-cell therapy, or a clinical trial.
Clinical trials are very important for Richter’s transformation. This is because it can be hard to treat, and doctors are still working to find better treatments. A clinical trial may give some people access to newer investigational treatment options. For more about clinical trials, please see here and here.
Your doctor may also talk with you about supportive care. Supportive care helps manage symptoms and side effects. This can include help with pain, tiredness, infection risk, nutrition, and emotional support.
Questions to Ask Your Doctor
You may want to ask:
- Do my symptoms suggest Richter’s transformation?
- What tests do I need?
- Do I need a PET/CT scan?
- Do I need a biopsy?
- What type of lymphoma do I have?
- Has the lymphoma spread to other parts of my body?
- What are my treatment choices?
- What is the goal of treatment?
- What side effects should I expect?
- Am I a good fit for a clinical trial? Which one(s)? Can you help me find one?
- Should I see a CLL/SLL or lymphoma specialist?
- Who should I call if my symptoms get worse?