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Richter's syndrome

This page tells you about Richter’s syndrome. It is a type of high grade non Hodgkin lymphoma. There is information about

 

Richter's syndrome

Richter’s syndrome is a type of high grade non Hodgkin lymphoma. It is a diffuse large B cell lymphoma. It is called diffuse because of the way the cells look under a microscope. Diffuse means spread out. Richter's syndrome 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 develops Richter's syndrome.  But it is a quickly developing cancer. People with Richter’s can become unwell quite suddenly.

 

Causes of Richter’s syndrome

We don’t know exactly what causes CLL to transform into Richter’s syndrome (RS). Researchers are looking into possible causes, including

The Epstein Barr virus

The Epstein Barr virus is very common, and most of us will have had 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 may trigger CLL cells to transform into high grade lymphoma cells.

Our genes

Researchers are also looking into how CLL cells develop into RS 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 RS cells.

We need more research to see exactly how genes affect the development of Richter’s. There is research looking at whether any particular gene causes the transformation.

 

Symptoms of Richter’s

Most people who develop Richter’s syndrome will have one or more of the following 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 and you can become ill quite suddenly.

 

Diagnosing Richter’s syndrome

Most people contact their doctor because they’ve developed new symptoms. Your doctor will examine you and arrange for you to have tests similar to those you would have had for CLL. These may include

Lymph node biopsy

To make a diagnosis of Richter’s syndrome your doctor will need to take some cells from one of your enlarged lymph nodes. A pathologist will then look at the cells under a microscope. For this test you’ll usually need to have one of the enlarged lymph nodes removed during a small operation, usually under general anaesthetic.

Blood tests

You may have various blood tests. These include a full blood count and a test to check your levels of an enzyme called lactate dehydrogenase (LDH). LDH is a normal substance in the blood, but it is at higher than normal levels in some types of cancer. LDH levels in the blood can go up if you have Richter’s syndrome. 

Other tests you might need are a

 

Treatments for Richter’s syndrome

The doctor who has been treating your CLL will continue to treat you if you develop Richter’s. The treatment is usually one of the following, or a combination of them

Chemotherapy

This is the most common treatment for Richter’s. Because Richter’s is similar to both acute leukaemia and lymphoma, your chemotherapy treatment may be the same as the

Monoclonal antibodies

Monoclonal antibodies (MABs) are a type of biological therapy. They are artificially made proteins that target specific proteins on cancer cells. Monoclonal antibodies are a fairly new treatment for cancer. Doctors often use the MAB drug rituximab with chemotherapy and steroids to treat Richter’s syndrome. Researchers in a trial called the CHOP-OR study are looking at whether a biological therapy similar to rituximab can make CHOP chemotherapy work better. The new drug is called ofatumumab (Arzerra). The study is for people who have just been diagnosed with Richter’s syndrome. 

People taking part in the CHOP-OR trial have ofatumumab with CHOP chemotherapy to get rid of the lymphoma (called induction treatment). They then have more ofatumumab on its own to try to stop the lymphoma coming back (called maintenance treatment). You can find out about trials for Richter's syndrome on our clinical trials database.

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. 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, so are only suitable for a few people. We need more research as it is too early to say how well this treatment works for Richter’s.

Radiotherapy

Radiotherapy is the use of radiation to treat cancer. You may have radiotherapy in combination with chemotherapy. You will only have radiotherapy

  • If Richter’s is affecting your brain or spinal cord
  • To control pain from enlarged lymph nodes
 

Coping with Richter’s syndrome

Coping with a rare cancer like Richter’s 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 may be trying to cope with the news that your cancer is aggressive and may 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 find detailed information about chronic lymphocytic leukaemia and non Hodgkin lymphoma on our website.

The coping with cancer section contains lots of information you may find helpful. If you want to find people to share experiences with on line, you could use CancerChat, our online forum. It is a place for anyone affected by cancer to share experiences, stories and information with other people who know what you are going through.

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Updated: 1 May 2013