Lymphoma is a type of blood cancer that develops when white blood cells called lymphocytes grow out of control. Lymphocytes are part of your immune system. They travel around your body in your lymphatic system, helping you fight infections. There are two types of lymphocyte: T lymphocytes (T cells) and B lymphocytes (B cells).
Some T-cell lymphomas develop in the skin. We have separate information on T-cell skin lymphomas.
T-cell lymphomas are rare. Around 700 people are diagnosed with T-cell lymphomas in the UK each year. They account for around 1 in 10 cases of non-Hodgkin lymphoma.
Most T-cell lymphomas develop from mature T cells. They usually affect adults, typically in people in their mid-60s. They are more common in men than in women. Occasionally, T-cell lymphoma can develop from immature T cells. This is known as T-cell lymphoblastic lymphoma. It tends to affect children and young adults.
In most cases, the cause of T-cell lymphomas is unknown. Research has shown that certain viral infections or health conditions can increase the risk of developing certain types of T-cell lymphoma.
- The human T-lymphotropic virus 1 (HTLV1) is linked with development of adult T-cell leukaemia/lymphoma (ATLL).
- Past infection with Epstein-Barr virus (EBV) is linked to the development of a range of lymphomas, including angioimmunoblastic T-cell lymphoma (AITL).
- Enteropathy-associated T-cell lymphoma (EATL) is linked with coeliac disease.
Remember that T-cell lymphomas are very rare and most people with these conditions do not develop lymphoma.
Symptoms of T-cell lymphomas can vary depending on the particular type of lymphoma you have and where it develops in your body.
The most common symptom of lymphoma is a swollen lymph node or nodes. Skin rashes or symptoms involving the skin are more common in some types of T cell lymphomas. Lymphoma that starts in the lymph nodes is called ‘nodal lymphoma’. However, many T-cell lymphomas develop outside the lymph nodes or in the organs – for example, in the liver, bone marrow, gut or skin. Lymphoma that starts outside the lymph nodes is called ‘extranodal’ lymphoma.
Extranodal lymphoma can cause many different symptoms, such as a swollen liver or spleen, a skin rash, or abnormal blood counts. These varied symptoms can make it difficult to diagnose T-cell lymphomas.
People with T-cell lymphomas often have symptoms known as ‘B symptoms’. Doctors will consider whether you have any B symptoms when they plan your treatment. The B symptoms are:
- night sweats
- unexplained weight loss.
T-cell lymphomas are rare and can be difficult to diagnose. Your doctor might consult specialist centres that have expertise in T-cell lymphoma. In some cases, your doctor might refer you to one of those centres.
It is important to find out exactly what type of T-cell lymphoma you have and what parts of your body are affected, so that your doctor can choose the best treatment for you. A number of different healthcare professionals are likely to be involved in your care.
T-cell lymphoma is usually diagnosed through a small procedure called a biopsy. A sample of tissue affected by the lymphoma, such as a swollen lymph node, is removed and examined by an expert lymphoma pathologist. The pathologist does tests on the tissue to find out what type of lymphoma you have.
You also have blood tests to:
- look at your general health
- check your blood cell counts
- make sure your kidneys and liver are working well
- rule out infections that could flare up when you have treatment.
If you are diagnosed with a T-cell lymphoma, you will have other tests to find out which areas of your body are affected by lymphoma. This is called staging. Staging usually involves having a PET/CT scan and a CT scan. Some people, particularly children or people with lymphoma in the central nervous system, may have an MRI scan. You might have a sample of your bone marrow cells taken (a bone marrow biopsy), to check if you have lymphoma cells in your bone marrow. Rarely, you might have a lumbar puncture or MRI scan to check if you have lymphoma in your brain or spinal cord (central nervous system).
It isn’t unusual for T-cell lymphoma to be at an advanced stage when it is diagnosed, as the lymphatic system goes all around the body. Although this sounds alarming, there are treatment options for advanced stage lymphoma.
Waiting for test results can be a worrying time. However, it is important for your medical team to know exactly what type of lymphoma you have and how it is affecting you. This helps them plan the most appropriate treatment for you.
There are many different types of T-cell lymphoma. They are sometimes grouped depending on whether they mainly affect lymph nodes (nodal T-cell lymphomas) or other parts of the body (extranodal T-cell lymphomas). We have dedicated pages outlining the symptoms and treatment for the most common types. They have complicated names based on the type of cell they develop from or the proteins they make.
- peripheral T-cell lymphoma not otherwise specified (PTCL-NOS)
- angioimmunoblastic T-cell lymphoma (AITL), follicular T-cell lymphoma (FTCL) and other T-cell lymphomas that develop from follicular helper T cells
- anaplastic large cell lymphoma (ALCL) including breast implant-associated T-cell lymphoma.
- extranodal NK/T-cell lymphoma, nasal type
- intestinal T-cell lymphomas, including enteropathy-associated T-cell lymphoma (EATL) and monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL)
- hepatosplenic T-cell lymphoma.
T-cell lymphomas that can be either nodal or extranodal include:
T-cell lymphoblastic lymphoma tends to affect younger people. We cover lymphoblastic lymphoma briefly on our page about lymphoma in children. Macmillan Cancer Support have more information on lymphoblastic lymphoma.
T-cell lymphomas that start in the skin behave differently, and are treated differently, from other types of T-cell lymphoma. We have separate information on T-cell skin lymphomas.
Your specific outlook depends on:
- the stage of your lymphoma
- the exact type of T-cell lymphoma you have
- your general health
- other individual factors.
Your medical team might use the results of your tests and other individual factors (for example, your age and your fitness levels) to calculate a score that helps predict how well you are likely to respond to treatment.
Your doctor is best placed to advise you on your outlook based on your individual circumstances.
It’s important to remember that most people do not find it helpful to look at survival statistics. Survival statistics can be confusing as they don’t tell you what your individual outlook is – they only tell you how a group of people with the same diagnosis did over a period of time. Remember that treatments are improving all the time and survival statistics are usually measured over 5 or 10 years after treatment. This means that statistics only tell you how people did in the past. Those people may not have received the same treatment that you will receive. It also includes the whole range of different outlooks from those who have a poor prognosis and to those who may be long-term survivors.
We have more detailed information about the treatment of particular types of T-cell lymphoma on our dedicated pages.
Lymphoma specialists and research scientists are investigating novel approaches to improve the treatments and outcomes for T-cell lymphomas. Several targeted drugs are being tested in clinical trials. Your doctor might ask you if you would like to take part in a clinical trial to help test new treatments.
- vincristine (also known as Oncovin®)
- prednisolone (a steroid).
It’s becoming more common for certain types of T-cell lymphoma to be treated with specific approaches. This might happen for people with anaplastic large cell lymphoma, extranodal NK/T-cell lymphoma and enteropathy associated T-cell lymphoma.
Some specialists might add a chemotherapy drug called etoposide to CHOP (CHEOP), or you might have a different chemotherapy regimen altogether. If you are not fit enough to have CHOP or CHEOP, you might be treated with less intensive chemotherapy, such as gemcitabine or bendamustine.
Your doctor might recommend a self (autologous) stem cell transplant for younger people with T-cell lymphoma who have responded well to initial chemotherapy and are well enough. For some types of T-cell lymphoma, this could give you a better chance of staying in remission (no evidence of lymphoma) after treatment.
When you finish treatment for T-cell lymphomas, you have regular follow-up appointments at the hospital. You have these appointments to check that:
- you are recovering well from treatment
- you have no signs of the lymphoma coming back (relapsing)
- you are not developing any late effects (side effects that develop months or years after treatment).
Typically, you have follow-up appointments every 2 to 3 months when your treatment first ends. This can vary depending on your particular circumstances and your hospital’s policy. Your appointments usually become less frequent over time.
People who stay in remission (no evidence of lymphoma) after treatment for T-cell lymphoma are usually followed up for at least 2 years after the end of their treatment. Some hospitals offer follow-up for 5 years or longer. If you have been treated as part of a clinical trial, you might be followed up for longer.
Contact your medical team if you develop any symptoms of lymphoma or if you have other concerns between your appointments. Your specialist might bring your appointment forward if they think they need to see you sooner.
After your follow-up period ends, your GP usually becomes your main point of contact if you have any concerns or notice anything unusual. Your GP should have a record of your diagnosis and all the treatment you’ve had. When you visit your GP for any reason, it is a good idea to remind them you have been treated for lymphoma in the past so they are aware of any health problems you may be at risk of.