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. 

What is lymphoma?

Lymphoma is a type of blood cancer. It affects white blood cells called lymphocytes Open a glossary item. It is also called a cancer of the lymphatic system Open a glossary item. 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.

Diagram of 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 Open a glossary item
  • fever which isn’t caused by an infection
  • night sweats
  • weight loss
  • sickness or tummy pain, caused by an enlarged spleen Open a glossary item 

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 lymph node biopsy Open a glossary item.  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 Open a glossary item
  • a PET-CT scan Open a glossary item

Treatment if you have Richter's syndrome

Your CLL specialist doctor will continue treating you if you develop Richter's syndrome. So you won't need to change doctors.

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 Open a glossary item
  • your general health 

The main treatments

The main treatments if you develop Richter's syndrome include:

  • chemotherapy Open a glossary item combined with immunotherapy, or on its own
  • a stem cell transplant Open a glossary item
  • 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).


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.


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.

For support and information, you can call the Cancer Research UK information nurses. They can give advice about who can help you and what kind of support is available. Freephone: 0808 800 4040 - Monday to Friday, 9am to 5pm.
  • Richter transformation of chronic lymphocytic leukaemia:a British Society for Haematology Good Practice Paper
    T Eyre and others
    British Journal of Haematology, 2022. Volume 196, Issue 4, Pages 864–870

  • Richter transformation in chronic lymphocytic leukemia/small lymphocytic lymphoma
    J Brown
    Accessed April 2024

  • Current trends in the management of Richter’s syndrome
    J.N Allan and R.R Furman
    International Journal of Hematologic Oncology, 2019. Volume 7, Issue 4, Pages 1-14

  • Approach to Richter transformation of chronic lymphocytic leukemia in the era of novel therapies
    M Khan and others 
    Annals of Haematology, 2018. Volume 97, Issue1, Pages 1-15

  • Histologic transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma
    R Agby and others 
    American Journal of Haematology, 2016. Volume 91, Issue 10, Pages 1036 - 1043

  • The information on this page is based on literature searches and specialist checking. We used many references and there are too many to list here. Please contact with details of the particular issue you are interested in if you need additional references for this information.

Last reviewed: 
19 Apr 2024
Next review due: 
19 Apr 2027

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