Richter's syndrome
Richter's syndrome can develop if you have chronic lymphocytic leukaemia (CLL). Doctors also call this Richter transformation. It is a rare complication of CLL.
Richter's syndrome means that the CLL changes (transforms) into a type of lymphoma. It usually transforms into a type of non-Hodgkin lymphoma (NHL). This is called diffuse large B cell lymphoma. Rarely, CLL can transform into Hodgkin lymphoma or other types of non-Hodgkin lymphoma.
This transformation can happen quickly, and you might become unwell quite suddenly. Your treatment will depend on the type of lymphoma you develop.
It is most common for Richter transformation to happen in someone with a CLL diagnosis. But doctors can sometimes diagnose it in someone who hasn't had a previous diagnosis of CLL.
What is lymphoma?
Lymphoma is a type of blood cancer. It affects white blood cells called 
. It is also called a cancer of the . This is because it starts in lymph nodes or other parts of the lymphatic system .
The lymphatic system is a system of thin tubes and lymph nodes that run throughout the body. Lymph nodes are bean shaped glands. The thin tubes are called lymph vessels or lymphatic vessels. Tissue fluid called lymph circulates around the body in these vessels. And it flows through the lymph nodes.
The lymphatic system is an important part of our immune system. It plays a role in fighting bacteria and other infections. And it tries to destroy old or abnormal cells, such as cancer cells.
The diagram shows the lymph vessels, lymph nodes and other organs. These make up the lymphatic system.
Non-Hodgkin lymphoma and Hodgkin lymphoma
There are 2 main types of lymphoma. These are Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL).
In Richter's syndrome, it is most common for CLL to change into a type of NHL. This type is called diffuse large B cell lymphoma (DLBCL). This is a fast growing (high grade) lymphoma. Around 90 out of 100 (around 90%) of Richter's syndrome change into DLBCL.
It is less common for CLL to transform into Hodgkin lymphoma. Around 10 out of 100 (around 10%) of Richter's syndrome change into Hodgkin lymphoma.
How common is Richter's syndrome?
Richter's syndrome is quite rare. Between 2 and 10 out of every 100 people (2-10%) with CLL develop Richter's syndrome.
Symptoms of Richter's syndrome
Most people who develop Richter’s syndrome have one or more of these symptoms:
- sudden swelling of your
lymph nodes - fever which isn’t caused by an infection
- night sweats
- weight loss
- sickness or tummy pain, caused by an enlarged
spleen
These symptoms usually develop quite quickly. You can become ill quite suddenly.
Getting diagnosed with Richter's syndrome
Most people contact their doctor because they have developed new symptoms. Your doctor examines you and arranges for you to have tests. These are usually similar to the tests you would have had for CLL.
You usually have a . A doctor removes part or all of the swollen lymph node. They send it to the laboratory for a specialist to look at it under a microscope. The results of this test shows if your CLL has transformed into a more aggressive type of lymphoma. And it helps the doctors find out what type of lymphoma it is.
Other tests you might have include:
- blood tests
bone marrow biopsy - a
PET-CT scan
Treatment if you have Richter's syndrome
Unfortunately, Richter's syndrome means your CLL has changed into a fast growing lymphoma. This can be difficult to treat. Your doctor will tell you more about what this means, and what your treatment options are.
Your treatment depends on:
- what type of lymphoma you develop
- whether you have changes (mutations) in certain
genes - your general health
The main treatments
The main treatments if you develop Richter's syndrome include:
chemotherapy combined with immunotherapy, or on its own- a
stem cell transplant - treatment as part of a clinical trial
Treatment if CLL transforms into diffuse large B cell lymphoma (DLBCL)
In Richter's syndrome, it is most common for CLL to change into a type of NHL. This type of NHL is called diffuse large B cell lymphoma (DLBCL).
Chemoimmunotherapy
You usually have chemotherapy combined with steroids and immunotherapy. Doctors call this chemoimmunotherapy. A common treatment is called R-CHOP. This includes the drugs rituximab, cyclophosphamide, vincristine and prednisolone.
You have the chemoimmunotherapy drugs on certain days. This is usually over 3 weeks. Each 3 week period is called a cycle of treatment. How many cycles you have depends on your situation.
Stem cell transplant
Your doctor might suggest you have a stem cell transplant after chemoimmunotherapy. This is intense treatment and you need to be fit enough to have it.
A stem cell transplant allows you to have very high doses of chemotherapy. You might have a transplant using:
- your own stem cells (autologous stem cell transplant)
- a donor’s stem cells (allogeneic stem cell transplant)
After high dose treatment, you have these stem cells into your bloodstream through a drip. This is the transplant. The cells find their way back to your bone marrow. You start making blood cells again and your bone marrow slowly recovers.
Treatment if CLL transforms into Hodgkin lymphoma
You usually have a combination of chemotherapy drugs. A common treatment for Hodgkin lymphoma is ABVD. This includes the drugs adriamycin (doxorubicin), bleomycin, vinblastine and dacarbazine.
Clinical trials
Your doctor might suggest you have treatment on a clinical trial. Researchers are looking at new treatments for people with lymphoma. Talk to your doctor or nurse if you want any more information about clinical trials.
Survival (prognosis) for Richter's syndrome
Richter's syndrome means your CLL has changed into a fast growing lymphoma. Unfortunately this can be more difficult to treat than other types of NHL or CLL. In general, the outlook (prognosis) for people with Richter's syndrome is poor.
The best person to talk to you about your outlook or prognosis is your doctor or specialist nurse. Not everyone wants to know. People cope differently with their lymphoma and want different information.
Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.
Coping
Coping with a rare syndrome can be especially difficult. You are unlikely to meet or hear about anyone else who has been in the same situation. So it can be harder to find out information and get the support you need. You might also be trying to cope with the news that this cancer is aggressive and can be difficult to treat.
It is important that you have enough information to enable you to make decisions about treatment. The doctors and nurses treating you should talk with you and answer any questions you have. Make sure that they tell you all your options and give you plenty of written information about all aspects of this cancer and its treatment.
