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Richter’s syndrome

Richter’s syndrome is a very rare type of high grade non Hodgkin lymphoma (NHL). Find out what causes it, how it is treated and more.

What it is

Richter’s syndrome is a type of high grade NHL called a diffuse large B cell lymphoma. Diffuse means spread out. It is called diffuse because of the way the cells look under a microscope. 

It is very rare. It starts as chronic lymphocytic leukaemia (CLL). Then sometimes the leukaemia cells get into the lymph nodes and start growing there. So in the advanced stage, CLL can change and become Richter's syndrome.

Fewer than 5 out of every 100 people (5%) with CLL develop Richter's syndrome.  But it is a cancer that develops quickly. People with Richter’s can become unwell quite suddenly.

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 doctor treating your CLL continues to treat you if you develop Richter's. The treatment is usually one or more of the following:

Chemotherapy

Chemotherapy is the most common treatment for Richter’s syndrome. Richter's syndrome is similar to both acute lymphoblastic leukaemia and non Hodgkin lymphoma.

You might have chemotherapy drugs used to treat NHL.

Or you might have the chemotherapy drugs used to treat acute lymphoblastic leukaemia. 

Biological therapy

Monoclonal antibodies are a type of biological therapy. They target specific proteins on cancer cells.

Doctors often use monoclonal antibody drug rituximab (Mabthera) in combination with chemotherapy drugs and steroids to treat Richter’s syndrome.

Chemotherapy and stem cell transplant

Stem cells are very early blood cells. Having a stem cell transplant means you have stem cells from a donor. The person who donates the stem cells is usually a brother or sister.

First you have very high doses of chemotherapy, sometimes with radiotherapy. This destroys both the cancerous and healthy cells in your bone marrow. After your chemotherapy treatment, the doctors give you the donor’s stem cells to replace your destroyed cells.

Stem cell transplant is an experimental way of treating Richter’s syndrome. While only a few people have had this treatment, for those people it appeared to work quite well. The disease was controlled for longer than for people having normal dose chemotherapy.

But stem cell transplants have serious side effects and complications and so are only suitable for a few people. We need more research to say how well this treatment works for Richter’s syndrome.

Radiotherapy

Radiotherapy uses radiation to treat cancer. You might have it in combination with chemotherapy. You only have radiotherapy if Richter’s syndrome is affecting your brain or spinal cord, or to control pain from enlarged lymph nodes.

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.

You can call the Cancer Research UK information nurses on freephone 0808 800 4040, from 9am to 5pm, Monday to Friday. They are happy to help. They can give advice about who can help you and what kind of support is available.
Last reviewed: 
01 May 2013
  • 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.

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