Richter Transformation

Richter transformation (Richter syndrome) is a rare complication of chronic lymphocytic leukemia (CLL). With Richter’s, CLL (a slow-growing cancer) becomes an aggressive cancer. It’s rare and only affects about 2 to 10 out of every 100 people with CLL.

In about 95% of Richter cases, CLL becomes diffuse large B-cell lymphoma (DLBCL). In about 5% of people with Richter transformation, CLL becomes Hodgkin lymphoma. Some people with CLL may develop DLBCL that may be unrelated to their CLL cells. This type usually has a better prognosis (outlook).

Given how aggressive Richter transformation is, it’s essential to get diagnosed and treated at the early signs of this rare complication. Your healthcare provider will evaluate you for Richter’s if they find rapid changes to your condition. They’ll review and adjust your treatment plan as needed.

Symptoms of Richter transformation

Symptoms usually start suddenly, get more intense without warning or progress rapidly. Richter transformation symptoms can happen at any time. But they usually start within the first few years of starting treatment for CLL.

You may have:

• Swelling in places where your lymph nodes are, like your neck, armpits and groin
• Abdominal pain or bloating
• Changes in appetite
• Fatigue
• Fever
• Night sweats
• Unexplained weight loss

Fever, night sweats and unexplained weight loss are known as “B symptoms” in lymphoma. They’re a sign of more active disease.

Richter transformation causes

Medical experts are still trying to learn why Richter syndrome happens. There doesn’t seem to be a single cause. Instead, people with this condition tend to have changes in their genes.

For example, it’s common to have changes (mutations) in specific genes that normally prevent tumors from forming. Changes also happen that speed tumor growth.

Research is ongoing to learn more about what these changes are and why they happen. The more experts learn about cancer cell changes, the more opportunities there are to find treatments that can target them.

How doctors diagnose this condition

If you have CLL, sudden swelling in your lymph nodes and new or worsening B symptoms may make your provider suspect Richter transformation. But you’ll need a biopsy to be sure. You’ll also likely need an imaging test called a PET-CT scan. It can show which areas in your body have more active cancer cells. This may mean CLL cells have evolved to Richter’s.

They’ll biopsy these areas with higher activity to check for transformed cancer cells.

How is it treated?

Treatment depends on your overall health and the changes in the cancer cells. Treatments include:

• Combination of chemotherapy and immunotherapy: A common treatment for DLBCL is R-CHOP. The goal of treatment is to send the cancer into remission. This means there are no signs or symptoms of cancer.
• Stem cell transplant: This treatment (also known as a bone marrow transplant) is the best option for potentially curing the cancer. You may get this treatment after cancer goes into remission. But it comes with a risk of complications, so not everyone’s a candidate for this intense treatment.
• Clinical trials: The best treatment option is often a clinical trial to access new therapies. This is because traditional treatments for Richter transformation are sometimes ineffective or too toxic.

New therapies

Researchers are studying new treatments that target the weaknesses in cancer cells. These targeted therapies destroy cancer cells. Researchers are also testing drugs that help your immune system fight cancer. New treatments include:

• Car T-cell therapy, which has helped some people achieve long-term remission
• Immunotherapy oral medications that target cancer cells more precisely

When should I see my healthcare provider?

Let your provider know right away if you’re living with CLL and develop new or worsening symptoms. Richter transformation is a sign that the cancer has become aggressive. You’ll likely need to discuss new treatment options with your provider.

Also, let them know if treatments cause side effects. It’s important to find a balance between keeping the cancer managed and maintaining your quality of life.

Work with your provider to find the treatment plan that works best at achieving this balance.

What can I expect if I have this condition?

Richter transformation can be hard to treat. So, it’s not always possible to send the cancer into remission. Even when it does, it may eventually come back. Some people stay in remission for a long time and become cured. But frequently, the life expectancy ranges from several months to a few years.

New therapies are constantly improving the life expectancy of people with cancers that are hard to treat. Talk to your healthcare provider about all of your options. This includes newer treatments that may be available if you take part in a clinical trial.

Can Richter syndrome go into remission?

Yes, but current treatment options may not send the cancer into remission for everyone. If your first treatment gets rid of the cancer, depending on your case, you may need a stem cell transplant to prevent the cancer from coming back.

Your healthcare provider will explain your treatment options based on your diagnosis, medical history and preferences.