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