Causes & Risk Factors of Richter's Transformation
It’s not known what causes some peoples CLL/SLL to go through Richter’s Transformation (RT) when other’s do not. It is very rare, and happens in less than 1 person out of every 10 (2-10%) with CLL/SLL.
There are some risk factors that increase your chances of Richter’s Transformation, but many people with these risk factors do not develop RT. Some risk factors include specific genetic mutations within your lymphoma cells. Mutations of the below genes can increase your risk.
- TP53
- NOTCH1
- CDKN2A
- MYC
As more research is done, new genetic links may be found. Ask your haematologist or oncologist about your genetic variations, and what these mean for your treatment options and outcomes.
Can early treatment for CLL/SLL prevent Richter's Transformation?
If you’ve been on Watch & Wait for your CLL/SLL you may wonder if having early treatment may have prevented Richter’s Transformation (RT). It’s a good question, and even some doctors and researchers have asked the very same question as part of research and clinical trials.
What they have found is that Watch and Wait does not increase your chance of going through RT. They have also found that people who have NEVER had treatment for their CLL/SLL may actually respond better to treatment for RT, than those who have previously had treatment.
What if I’ve had treatment, was that a mistake?
It’s important to remember that Richter’s Transformation is a very rare complication of CLL/SLL, and most people will not go through RT. For this reason, it is unreasonable to not have needed treatment for CLL/SLL just in case you progress to RT.
Your haematologist or oncologist will have looked at your individual circumstances including your biopsy and genetic test results, your symptoms and your overall wellbeing when they made a decision to offer you treatment. Based on all this information, they will have determined that the benefits of having treatment were a better option than the risk of you not having treatment.
When does Richter's Transformation happen?
Richter’s transformation can happen at any time after your CLL/SLL diagnosis however, it is more common within months to 2 years after your initial diagnosis. For a very small number of people, it may already be taking place when you are first diagnosed with CLL/SLL.
Symptoms of Richter's Transformation
You may have never had symptoms of your CLL/SLL, or if you did they may have occurred over time and been less obvious. However, when you have Richter’s Transformation you are very likely to have symptoms.
Some of the more common symptoms you may experience include:
- Swollen lymph nodes that come up quickly without infection and do not go down within 2 weeks.
- B-symptoms – these include fevers without infection and/or weight loss without trying, and/or drenching night sweats.
- General, but rapid deterioration in your physical health.
- Increased and severe fatigue.
- Other general symptoms of lymphoma – see images below.
Blood tests
As well as the above symptoms, there may be signs in your blood tests that show you may be going through Richter’s transformation. Blood tests may show high levels of calcium and/or lactate dehydrogenase (LDH).
Diagnosis of Richter's Transformation
You will need more biopsies, much like you had at the time you were first diagnosed with CLL/SLL. These will include biopsies of your swollen lymph nodes, blood tests and a bone marrow biopsy. Your haematologist or oncologist will also order cytogenetic tests to be done on the biopsies to learn more about the genetic mutations you now have. These mutations may be different to the ones you had when you were first diagnosed with CLL/SLL. Results from these tests will help your haematologist to develop a treatment plan to offer you.
Treatment for Richter's Transformation
Many people with a primary diagnosis of DLBCL and Hodgkin Lymphoma respond very well to treatments and have a high chance of cure or long-term remission. However, when these subtypes occur as a result of Richter’s Transformation, they can be more difficult to treat and not always respond as well to the standard treatments. This is due to underlying genetic mutations that can make the lymphoma resistant to the effects of chemotherapy.
Considerations when deciding best treatment options
Due to the rarity of Richter’s Transformation and the variations within the lymphoma cells there is currently no standard treatment. Your haematologist or oncologist will look at your biopsy and genetic results to determine the best treatment options for you. Some things they will consider when choosing the best treatment for you will include:
- If you have ever had treatment for your CLL/SLL in the past.
- What type of treatment you had.
- Whether the Richter’s Transformation has developed into DLBCL or Hodgkin Lymphoma.
- Genetic mutations and proteins found in/on your lymphoma cells.
- Your age and overall health.
- Any other illnesses you have, and medication you are taking.
- Your personal preferences after getting all the information you need.
Types of treatment you may be offered
Treatments you may be offered, based on whether your Richter’s Transformation resulted in DLBCL or Hodgkin Lymphoma are listed in the table below.
Possible treatment options | |
Diffuse Large B-cell Lymphoma (DLBCL) secondary to Richter’s Transformation | Hodgkin Lymphoma secondary to Richter’s Transformation |
Chemotherapy alone:
Chemoimmunotherapy (chemotherapy with a monoclonal antibody):
Targeted therapy with or without the above chemotherapy or chemoimmunotherapy
Bispecific antibody
Cellular or biologic therapies:
| Chemotherapy alone:
Conjugated monoclonal antibody with or without above chemotherapy
Immune checkpoint inhibitor with or without above chemotherapy
Cellular therapy:
|
Clinical Trials
Clinical trials are an important way to find new treatments, or combinations of treatments to improve outcomes for patients with Richter’s Transformation. They also can offer you the opportunity to try new types of treatment that have not previously been approved for your type of lymphoma.
Ask your doctor if there are any clinical trials for people with Richter’s Transformation that you could participate in.
Prognosis
A prognosis is the expectation of how a disease will behave, and how it may affect you and your life.
Richter’s Transformation (RT) is a major change that can have devastating consequences. There is no known cure for Richter’s Transformation and treatment options are often based on improving your symptoms and giving you extra time, rather than achieving a remission or cure.
Clinical trials and research are ongoing and hope to improve outcomes for people with Richter’s Transformation in the near future. However, currently there are limited options available, and most people will unfortunately have a significantly shortened life-span.
Your haematologist is the best person to give you information on your individual prognosis, and how long you may live after Richter’s Transformation however sadly, most people will live for less than 2 years after RT.
Emotions
- shock
- anger
- sadness
- disbelief
- loneliness
- fear
- acceptance.
Get support
It is a good idea to consider how you and your family will cope with the emotions and distress after RT diagnosis. There is lots of support available.
Lymphoma Australia Nurses
Our Lymphoma Nurses are available Monday to Friday 9am-4:30pm (Brisbane time) to talk to and your loved ones and can offer support and advice. They can also provide you with information on different services available to help you and your loved ones though this time. You can contact them by phoning 1800 953 081 or by email on nurse@lymphoma.org.au.
Your GP (local doctor)
Your GP is a great place to start. If you don’t already have a regular GP, now is the time to find one. They will be a valuable source of support and can coordinate all your care needs.
A GP management plan allows you to access up to 5 allied health consults with no, or very little out of pocket cost to you. These can include physiotherapists, exercise physiologists, occupational therapists and more.
To learn more about what is covered by allied health, please see the below link.
Allied health professions – Allied Health Professions Australia (ahpa.com.au)
Everyone affected by cancer should have a mental health plan and if you have life-limiting cancer such as with RT, it is even more important. They are also available to your family members and provide you with 10 visits or telehealth appointments with a psychologist. The plan also helps you and your GP discuss what your needs will be as you near the end of your life, and make a plan to cope with the reality of your diagnosis, and create plans to make the most of the rest of your life, as well as your end of life plans.
Find more information on what mental health care is available here Mental health care and Medicare – Medicare – Services Australia.
Advance Care Planning
Advance Care Planning Australia states:
Advance care planning gives you the opportunity to think about, discuss and record your preferences for the type of care you would like to receive, and the outcomes you would consider acceptable. Advance care planning helps to ensure your loved ones and health providers know what matters most to you and respect your treatment preferences."
An advance care plan is something everyone in Australia should consider completing. If you do not already have one, having RT is a good reason to do one. It allows you to still have control over decisions affecting your health care, and can take the burden off you and your loved ones if you become too weak to actively make decisions.
Click the link below to learn more about Advance Care Planning and to create your individual plan.
Palliative Care
Palliative care services include different health professionals including doctors, nurses and allied health. Most palliative care services are covered through Medicare, so you will not have to pay for them to be involved in your care. They are experts at managing symptoms of your disease and side-effects of treatment, as well as providing end of life care.
Research shows that people connected with palliative care often live a little longer than others with the same disease without palliative care. People connected with palliative care also report having much better quality of life as they near the end of their life.
You can find more information on Palliative Care and how they can help you by clicking the link below.
Ideas for celebrating your life
Some people find it easier to cope with a terminal illness by celebrating life rather than worrying about death. This may be easier said than done, and it is normal to worry about the end. But if you have other things to look forward to or focus on, you can still take control of your life and enjoy special times.
Some ideas you may like to think about include:
- Plan and go on a trip while you can.
- Declutter your life and your home. Surround yourself with only things that bring joy, truth and happiness.
- Have a living wake – don’t wait until your funeral to get everyone together, bring them together when you can still enjoy their company and hear what they have to say about you.
- Go for walks or sit at the beach or in nature.
- Write love letters or make a video recording to all the important people in your life.
- Have a photo day with family and friends making memories with you.
- Write a book, song or poem about your life to leave your loved ones.
- Make a playlist of songs that have meant something to you at different stages and times throughout your life.
Summary
- Richter’s Transformation is a rare and serious complication of CLL/SLL.
- It occurs when the CLL or SLL cells transform into a different type, and aggressive subtype of lymphoma such as Diffuse Large B-cell Lymphoma (DLBCL) or Hodgkin Lymphoma.
- It is not known why some people progress to RT while others don’t, but there are some genetic variations that can increase your risk.
- Treatment for RT is aimed at managing your symptoms rather than cure or remission.
- There is currently no know treatment that can achieve cure or remission in RT.
- Sadly, most people live less than 2 years after RT.
- Getting the right support and completing an Advance Care Plan can help you maintain some control over your health care during this time.
- Lymphoma Care Nurses are here to support you. You can contact us by clicking the Contact Us button at the bottom of the screen.
