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Richter’s syndrome

Richter’s syndrome is a very rare type of high grade non-Hodgkin lymphoma. 

What is it?

Richter's syndrome is a rare type of non-Hodgkin lymphoma (NHL). NHL is a cancer of the lymphatic system.

People who develop Richter's syndrome have a type of blood cancer called chronic lymphocytic leukaemia (CLL). The leukaemia cells get into the lymph nodes and start growing there. So in the advanced stage, CLL can change and become a high grade lymphoma. This change or transtion is called Richter's syndrome. 

With Richter's syndrome the CLL usually changes into a high grade (fast growing) NHL called diffuse large B cell lymphoma.

This type of cancer grows quickly, and people might become unwell quite suddenly. Between 2 and 10 out of every 100 people (2-10%) with CLL develop Richter's syndrome.

Causes

We don’t know exactly what causes CLL to transform into Richter’s syndrome. Researchers are looking into possible causes, including the Epstein Barr virus and genes.

Epstein Barr virus

The Epstein Barr virus is very common. Most of us have it at some point in our lives.

Normally it causes glandular fever. But sometimes you have it without any symptoms. So you can have it without realising.

Researchers have found that the Epstein Barr virus might trigger CLL cells to transform into high grade lymphoma cells.

Genes

Researchers are also looking into how CLL cells develop into Richter’s syndrome cells, and which genes are involved in this change.

Several genes cause the CLL cells to multiply. Researchers think that as the cells multiply, their genes undergo further changes which transform them into Richter’s syndrome cells. They are looking at whether any particular gene causes the transformation.

Symptoms

Most people who develop Richter’s syndrome have one or more of these symptoms:

  • sudden swelling of their lymph nodes
  • fever which isn’t caused by an infection
  • night sweats
  • weight loss
  • sickness or abdominal pain if your spleen is enlarged

These symptoms usually develop quite rapidly. You can become ill quite suddenly.

Getting diagnosed

Most people contact their doctor because they have developed new symptoms. Your doctor examines you and arranges for you to have tests similar to those you would have had for CLL. These might include

  • testing your lymph nodes (a biopsy)
  • blood tests.
  • bone marrow biopsy
  • CT scan.

Treatment

The medical team treating your CLL continue to treat you if you develop Richter's. 

Chemotherapy and immunotherapy

You usually have a combination of drugs to treat Richter’s syndrome. The combination depends to some extent on the type of lymphoma your CLL has changed into. Or you might also have other drugs normally used to treat another type of blood cancer called acute lymphoblastic leukaemia. 

The combination includes some chemotherapy drugs and a steroid, such as prednisolone or dexamethasone. You might also have a targeted immunotherapy drug, such as rituximab. These drugs target specific proteins on lymphoma cells and help the immune system to pick out these cells and kill them. 

For Richter's syndrome that has changed into diffuse large B cell lymphoma you might have a combination of drugs such as:

  • R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone)

Stem cell transplant

Between 10 and 15 out of every 100 people (10-15%) with Richter's syndrome have high dose chemotherapy, followed by a stem cell transplant. You have to be fairly fit and well to have this treatment as it is very intensive. 

Before your high dose chemotherapy, your team either collects:

  • your stem cells
  • a donor's stem cells 

After high dose treatment, you have these stem cells into your bloodstream through a drip (the transplant). The cells find their way back to your bone marrow. You start making blood cells again and your bone marrow slowly recovers. 

Radiotherapy

Radiotherapy uses radiation to treat cancer. You might have it with chemotherapy in preparation for your stem cell transplant. 

Coping

Coping with a rare cancer like Richter’s 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 on freephone 0808 800 4040, from 9am to 5pm, Monday to Friday. They can give advice about who can help you and what kind of support is available.
Last reviewed: 
06 Feb 2018
  • Approach to Richter transformation of chronic lymphocytic leukemia in the era of novel therapies

    M Khan and others 

    Annals of Haematology, 2018. Volume 97, 97(1) pages 1-15

  • Histologic transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma

    R Agby and others 

    American Journal of Haematology, 2016. Vol.ume 91, No.10, pages 1036 - 1043

  • Richter syndrome: pathogenesis and management

    D Rossi and others 

    Seminars in Oncology, 2016. Volume 43(2) pages 311-9

  • Richter's transformation in chronic lymphocytic leukemia
    ​AM Tsimberidou and MJ Keating
    Seminars of Oncology, 2006 Apr; Volume 33, Issue 2, Pages 250-6

  • Richter's syndrome: biology and therapy
    KW Yee and others
    Cancer Journal. 2005 May-Jun, Volume 11, Issue 3, Pages 161-74.

  • 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 patientinformation@cancer.org.uk with details of the particular issue you are interested in if you need additional references for this information.

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