T-cell lymphomas

What are T-cell lymphomas?

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).

Lymphomas can be grouped as Hodgkin lymphomas or non-Hodgkin lymphomas, depending on what the lymphoma cells look like under a microscope. 

T-cell lymphomas are non-Hodgkin lymphomas that develop from T lymphocytes.

Some T-cell lymphomas develop in the skin (cutaneous lymphoma). We have separate information on skin T-cell lymphomas.

T-cell lymphomas are rare. Around 1,000 people are diagnosed with T-cell lymphomas in the UK each year. 

Back to top

Diagnosis and staging of T-cell lymphomas

T-cell lymphomas are rare and can be difficult to diagnose. Your doctor will often 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 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. This might need to be repeated if more information is needed to make a diagnosis.

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. Some people, particularly children or people with lymphoma in the central nervous system (brain, spinal cord and/or eyes), 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 central nervous system.

T-cell lymphoma is often at an advanced stage when it is diagnosed, as the lymphatic system is found everywhere in 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.

Back to top

Types of T-cell lymphoma, their symptoms and treatment

There are many different types of T-cell lymphoma. They have complicated names based on the type of cell they develop from or the proteins they make, what the lymphoma cells look like and where in the body the lymphoma is.

T-cell lymphomas include:

• peripheral T-cell lymphoma not otherwise specified (PTCL-NOS)
• nodal T-follicular cell lymphoma (nTFHL), including angioimmunoblastic, follicular and NOS types
• anaplastic large cell lymphoma (ALCL) including breast implant-associated T-cell lymphoma.
• adult T-cell leukaemia/lymphoma (ATLL)
• intestinal T-cell lymphomas, including enteropathy-associated T-cell lymphoma (EATL) and monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL)
• extranodal NK/T-cell lymphoma, nasal type
• hepatosplenic T-cell lymphoma

The following two types of T-cell lymphoma are not covered on this page as they are treated differently:

• T-cell lymphoblastic lymphoma. This type tends to affect younger people. It is similar to acute lymphoblastic leukaemia (ALL), and is treated in the same way. Leukaemia Care provides more detailed information about ALL.
• T-cell skin lymphoma (cutaneous lymphoma). These T-cell lymphomas behave differently, and are treated differently, from other types of T-cell lymphoma. We have separate information on T-cell skin lymphomas. 

Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS)

PTCL-NOS includes all T-cell lymphomas that don’t fit into any other category. 

PTCL-NOS is the most common group of T-cell lymphomas, accounting for around 1 in 3 cases. It can occur at any age, but it is more common in people in their 60s. It tends to affect more men than women. Most people have advanced stage lymphoma when they are diagnosed.

The lymphomas within this group can be quite different from each other. However, they might share some common symptoms. The most common symptom people with PTCL-NOS might have is a swollen lymph node or nodes. These can develop anywhere in the body, but the most common places are the neck, armpit or groin. 

PTCL-NOS is also commonly found outside the lymph nodes (extranodal sites), leading to different symptoms.

• If the bone marrow (the spongy tissue in the centre of bones where blood cells are made) is affected, it can lead to anaemia (low red blood cells) and thrombocytopenia (low platelet levels).
• If the liver and spleen are affected, they might be enlarged, causing bloating or tummy (abdominal) pain.
• If you have lymphoma in your gut, it can cause pain, sickness, diarrhoea or vomiting.
• If the skin is affected, it can cause red, itchy patches.
• If you have lymphoma in your chest, you might feel short of breath, develop a cough or have pain or a feeling of pressure in your chest.

Fevers, night sweats and unexplained weight loss (known as ‘B symptoms’) are also common in people with PTCL-NOS.

Treatment of PTCL-NOS

Whilst PTCL-NOS is the most common type of T-cell lymphoma, it is rare. This makes it difficult for doctors to determine which treatment will give you the best outcome. Your doctor might ask you if you would like to take part in a clinical trial to help find out what the best treatment is for PTCL-NOS. If you don’t want to take part in a clinical trial, or if there isn’t one that is suitable for you, you are likely to be treated with chemotherapy. The most common chemotherapy regimen used for PTCL-NOS is CHOP:

• cyclophosphamide
• doxorubicin (or hydroxydaunorubicin)
• vincristine (also known as Oncovin®)
• prednisolone (a steroid).

If you respond to chemotherapy and you are well enough, your doctor might discuss the option of having a self (autologous) stem cell transplant. This decision needs to be made for each individual taking into account the possible benefits and risks.

Nodal T-follicular cell lymphoma (nTFHL) 

nTFHL develops from a type of white blood cell called a ‘follicular helper T cell’. There are three main types: angioimmunoblastic-type, follicular-type and NOS (not otherwise specified)-type.

nTFHL accounts for about 1 in every 4 cases of T-cell lymphoma. Around 250 people are diagnosed with nTFHL in the UK each year. These types of lymphoma often affect older people, but may also occur in young people. Men are more commonly affected.

Scientists currently don’t know what causes nTFHL. These types of T-cell lymphoma have been linked to infections including viruses called Epstein-Barr virus (EBV) and human herpesvirus 6 (HHV-6). However, this potential relationship between having these infections and nTFHL remains unclear and further research is ongoing. Ageing has also been suggested as a strong factor due to an accumulation of errors in blood cells over time that might increase the risk of cancer.

Most people with nTFHL present to their doctor with advanced stage lymphoma (stage 3 or 4). 

The most common symptoms of nTFHL are:

• fever, night sweats and unexplained weight loss (‘B symptoms’)
• swollen lymph nodes, usually in several places
• a swollen liver and spleen, which might make you feel bloated or cause tummy (abdominal) pain
• itching
• skin rash.

nTFHL often affects your bone marrow (the spongy tissue in the centre of your bones where blood cells are made), which can lead to low levels of red blood cells (anaemia) and platelets (thrombocytopenia). Occasionally, there might be a build-up of fluid around the lungs (pleural effusion), which can cause breathing difficulties.

The lymphoma can also affect how well your immune system works so you might find it harder than usual to fight off infections.

In some people with nTFHL, abnormal immune cells might produce too many antibodies (also known as ‘immunoglobulins’). Antibodies are proteins that B cells usually make to help you fight off infection. If you have nTFHL, you might make abnormal antibodies that damage healthy cells. This is called an ‘autoimmune reaction’. It can cause a variety of symptoms, including:

• a low red blood cell count (autoimmune haemolytic anaemia), which might make you feel tired or short of breath
• low platelet levels (immune thrombocytopenia), which might make you bleed or bruise more easily than normal
• painful, swollen joints
• inflammation of your blood vessels, which can cause a range of symptoms including skin rashes
• thyroid problems.

Treatment of nTFHL

Treatment of nTFHL can include treatment with steroids or chemotherapy.

Several treatments 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 and to find out what the best treatment is for nTFHL.

If you don’t want to take part in a clinical trial, or if there isn’t one that is suitable for you, you are likely to be treated with chemotherapy. 

The most common chemotherapy regimen used for nTFHL is CHOP, which consists of: 

• cyclophosphamide
• hydroxydaunorubicin
• vincristine (also known as Oncovin®)
• prednisolone.

If you respond to chemotherapy and you are well enough, your doctor is likely to discuss the option of having a self (autologous) stem cell transplant. This decision needs to be made for each individual taking into account the possible benefits and risks.

Some people with nTFHL have a high number of abnormal B cells in their body. If this is the case, you might have antibody treatment such as rituximab as well as chemotherapy.

Anaplastic large cell lymphoma (ALCL)

ALCL is rare. Each year, fewer than 200 people are diagnosed with ALCL in the UK.

There are four main types of ALCL. They have complicated names based on their features and the types of proteins they make:

• ALK-positive ALCL (also known as ALK+ ALCL) is the most common type of ALCL. The abnormal T cells have a genetic change (mutation) that means they make large amounts of a protein called ‘anaplastic lymphoma kinase’ (ALK). ALK can be detected with tests on the surface of the tumour cell – the T-cells test positive for ALK. This is a fast-growing (high-grade) lymphoma. ALK-positive ALCL usually affects children and young adults, commonly in their 30s. It affects three times more males than females. The other 3 types of ALCL are all ALK-negative.
• ALK-negative ALCL (also known as ALK- ALCL) accounts for around 3 in every 10 cases of ALCL. The abnormal T cells do not make the ALK protein – they test negative for ALK. This is a fast-growing (high-grade) lymphoma. ALK-negative ALCL tends to affect older adults, typically around 40 to 65 years old. It is slightly more common in men than women.
• Breast implant-associated ALCL develops extremely rarely following silicone breast implantation and appears to be more common in textured rather than smooth implants. It typically develops 8 to 10 years after having the implant but it can develop sooner. Although it develops in the breast, it is not a type of primary breast cancer. It is usually a slow-growing (low-grade) lymphoma and is thought to be caused by an inflammatory reaction initiated by the breast implant.
• Primary cutaneous ALCL is a low-grade T-cell lymphoma that develops in the skin. We have more information on this type of ALCL on our page about T-cell skin (cutaneous) lymphoma.

If you are under 25 and have been diagnosed with ALCL, or if you have a child who has been diagnosed with ALCL, you might find our information on lymphoma in children and young people useful.

People with ALK-positive and ALK-negative ALCL typically have swollen lymph nodes and B symptoms (fevers, night sweats and weight loss). ALCL is also commonly found outside the lymph nodes (extranodal areas), where it can cause many different symptoms.

• Lymphoma affecting the gut (gastrointestinal system) might cause bloating, pain, sickness, diarrhoea or vomiting.
• If you have lymphoma in your chest, you might feel short of breath, develop a cough or have pain or a feeling of pressure in your chest.
• Your bone marrow (the spongy tissue in the core of bones where blood cells are made) might be affected, which can lead to anaemia (low red blood cells) and thrombocytopenia (low platelet levels).
• You might develop a rash.

Most people with ALK-positive and ALK-negative ALCL are diagnosed at an advanced stage (stage 3 or 4), which means the lymphoma affects several parts of the body.

People with breast implant-associated ALCL typically develop a build-up of fluid or a lump around the implant. It can be uncomfortable. This type of ALCL does not usually spread outside the affected breast. It is usually diagnosed at an early stage (stage 1 or 2).

Treatment of ALK-positive and ALK-negative ALCL

ALCL is rare. This makes it difficult to determine which treatment gives the best outcome. Your doctor might ask you if you would like to take part in a clinical trial to help find out what the best treatment is for ALCL.

If you don’t want to take part in a clinical trial, or if there isn’t one that is suitable for you, you are likely to be treated with chemotherapy in combination with a targeted drug called brentuximab vedotin (BV).

The most common chemotherapy regimen used for ALCL is BV-CHP:

• brentuximab vedotin
• cyclophosphamide
• doxorubicin (or hydroxydaunorubicin)
• prednisolone (a steroid).

The number of cycles and the exact type of chemotherapy you have depends on several factors like your age, the stage of your lymphoma and whether it is ALK-positive or ALK-negative. Some people with early-stage ALCL might also have radiotherapy to the affected area.

If you respond to chemotherapy, your doctor might discuss the option of having a self (autologous) stem cell transplant. This decision needs to be made for each individual taking into account the possible benefits and risks.

Treatment for breast implant-associated ALCL

If you have breast implant-associated ALCL, you have the implant, and any lumps or fluid, removed by surgery. This might be all the treatment you need. You are then followed up carefully to make sure all the lymphoma has been removed. If the lymphoma is more widespread, you are likely to have chemotherapy, usually with a regimen called BV-CHP which is the anti-body-drug conjugate brentuximab vedotin combined with the chemotherapy regimen CHP.

Adult T-cell leukaemia/lymphoma (ATLL)

ATLL is very rare. Only around 50 people are diagnosed with it in the UK each year. ATLL can affect men and women of any age but it most commonly develops around the age of 50.

ATLL only affects people who have a virus called ‘human T-lymphotropic virus type 1’ (HTLV-1). HTLV-1 infection is rare in the UK, but it is very common in some parts of the world. These include southern Japan, the Caribbean Basin, Central and South America, Iran, Romania and parts of Africa.

HTLV-1 infection is spread from person-to-person through breastfeeding, unprotected sex, sharing contaminated needles or equipment, or infected blood transfusions or organ transplants. People who have HTLV-1 may not know they are infected. 

Only around 1 in 20 people with HTLV-1 develop ATLL, often several decades after becoming infected with HTLV-1. Most people with HTLV-1 do not develop lymphoma. Only carriers with a high level of virus in their blood (a ‘high viral load’) are at risk of developing ATLL. Your viral load can be measured by a blood test.

ATLL can occur within families. It can spread within families through mother-to-child breast feeding. If you have ATLL, your brothers and sisters, children and parents should be tested for HTLV-1 infection to monitor their risk. The is a specialist service in the UK to diagnose and care for people living with HTLV-1, ask your medical team for details.

There are four main types of ATLL: 

• Lymphoma-type ATLL is the most common type of ATLL. It is called ‘lymphoma-type’ because you have abnormal white blood cells in your lymphatic system but not in your blood.
• Acute (leukaemic) ATLL is called ‘leukaemic’ because you have abnormal white blood cells (‘leukocytes’) in your blood and your lymphatic system. Acute ATL is more likely to affect other organs, including the nervous system.
• Chronic ATLL is called ‘chronic’ because it develops slowly over a long time. Chronic ATLL affects the blood and the lymph nodes.
• Smouldering ATLL is called ‘smouldering’ because it can go on for a long time without causing many problems. Smouldering ATLL typically affects the skin and occasionally the lungs. This can be an early stage lymphoma that can develop into one of the other types.

Lymphoma-type ATLL and acute ATLL are high-grade lymphomas. Symptoms can develop quickly. Symptoms of these types of ATLL include:

• swollen lymph nodes
• a swollen liver and spleen, which might make you feel bloated or cause tummy (abdominal) pain
• a skin rash, which can vary from widespread, itchy red patches to lumps or skin tumours that can break down (ulcerate) and scab over
• high levels of calcium in your blood, which can cause sickness, diarrhoea, constipation, headaches, weeing more than usual, or feeling thirsty, weak, tired or confused
• fever, night sweats and weight loss (‘B symptoms’)
• abdominal (tummy) symptoms
• getting more infections than usual, or having difficulty shaking off infections.

Some people with ATLL develop skin tumours that grow very quickly. This is sometimes called ‘cutaneous lymphoma-type ATLL’. This is different from the common types of skin cancer, and is related to the ATLL.

In around 1 in 10 people, acute or lymphoma-type ATLL can affect your bones, which might make your bones ache or break more easily than normal. Occasionally, ATLL can spread to your brain or spinal cord (central nervous system). 

Chronic and smouldering ATLL are low-grade lymphomas. 

• Smouldering ATLL often causes a skin rash but some people don’t have any symptoms at all. You might just have a blood test that shows abnormal T cells in your bloodstream.
• The most common symptoms of chronic ATLL are a skin rash, swollen lymph nodes or a swollen liver or spleen. Symptoms are usually mild.

Treatment of lymphoma-type ATLL and acute ATLL

Lymphoma-type ATLL and acute ATLL are fast-growing and need to be treated straightaway. It is common for these types of lymphoma to come back (relapse) after treatment.

There is no standard treatment for these types of ATLL. Your doctor might ask you if you would like to take part in a clinical trial to help find out what the best treatment is for ATLL.

If you don’t want to take part in a clinical trial, or if there isn’t one that is suitable for you, you are likely to be treated with strong chemotherapy. Some people also have antiviral drugs such as zidovudine and interferon-alfa to control their HTLV-1 infection. You have blood tests to check for high calcium levels so you can be treated quickly if it develops.

The most common chemotherapy regimens (combination of drugs) used to treat acute or lymphoma-type ATLL are:

• CHOP: cyclophosphamide, hydroxydaunorubicin, vincristine (also known as Oncovin®) and prednisolone
• CHEOP: CHOP plus etoposide

If your doctor thinks you are at high risk of your lymphoma affecting your central nervous system (CNS), you might have additional chemotherapy drugs to try to prevent this. This is called CNS prophylaxis. 

If you respond to chemotherapy and you are well enough, your doctor is likely to recommend that you have a donor (allogeneic) stem cell transplant. This can give you a better chance of staying in remission (no evidence of lymphoma). 

Treatment of chronic ATLL and smouldering ATLL

If you have chronic ATLL or smouldering ATLL and you don’t have any troublesome symptoms, you might not need any treatment at first. Instead, your doctor monitors you closely. This is called active monitoring (‘watch and wait’). 

If there is a risk of chronic or smouldering ATLL becoming more aggressive, your doctor is likely to offer you treatment. They might ask you if you would like to take part in a clinical trial. If you don’t want to take part in a clinical trial, or if there isn’t one that is suitable for you, you are likely to be treated with antiviral drugs such as zidovudine and interferon-alfa. These help to fight HTLV-1 infection and slow the growth of ATLL.

If you have skin symptoms, you might also have skin-directed (topical) treatments or light therapy.

If your ATLL changes to a faster-growing type, continues to get worse even with treatment or you have skin tumours that are growing, you are treated the same way as lymphoma-type or acute ATLL.

Intestinal T-cell lymphomas

Intestinal T-cell lymphoma is a rare type of fast-growing (high-grade) non-Hodgkin lymphoma that grows in your small bowel (gut or intestine). Intestinal T-cell lymphomas usually affect older people, often in their 60s.

There are two types of intestinal T-cell lymphoma:

• Enteropathy-associated T-cell lymphoma (EATL) is the most common type of intestinal T-cell lymphoma. ‘Enteropathy’ means a ‘disease of the intestines’. EATL tends to affect people of northern European origin. It is strongly linked to an autoimmune condition called coeliac disease, which affects around 1 in 100 people. Coeliac disease develops when your immune system makes antibodies in reaction to the gluten in the food you eat. These antibodies mistakenly attack your small bowel, so you can’t absorb food properly. Coeliac disease is usually controlled by following a gluten-free diet. EATL only develops in people who have coeliac disease, although some people might not know they have coeliac disease until they are diagnosed with EATL. Most people who have coeliac disease do not develop EATL, especially if they follow a gluten-free diet.
• Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is less common, and accounts for around 1 in every 5 cases of intestinal T-cell lymphoma. It is called MEITL because the abnormal T cells all look the same (they are ‘monomorphic’) and it grows in the lining of your intestine (your intestinal ‘epithelium’). MEITL is not linked to coeliac disease. It is more common in people of Asian or Hispanic origin.

The most common symptoms of intestinal T-cell lymphoma are bowel and stomach problems. You might have:

• tummy (abdominal) pain
• weight loss
• diarrhoea, which might have blood in it
• fatigue
• an itchy rash.

You might be malnourished (not getting enough nutrients) if you are not absorbing food properly. You might also have ‘B symptoms’ (fevers, night sweats and unexplained weight loss). Very occasionally, you might have a blockage in your bowel or a burst bowel (‘perforated’ or ‘ruptured’ intestine). This is very serious and requires urgent surgery.

Intestinal T-cell lymphoma can be difficult to diagnose because the symptoms are similar to lots of other conditions that affect the bowel, including coeliac disease itself. The bowel can also be difficult to see on standard scans.

You usually need an endoscopy to diagnose intestinal T-cell lymphoma. This is an examination of your bowel using a thin tube that is inserted into your body through your mouth or bottom (anus). Tools can be passed through the tube to take samples of your small bowel (a small bowel biopsy) to be examined under a microscope. The NHS website has more information about endoscopy.

Treatment of intestinal T-cell lymphomas

Intestinal T-cell lymphoma is difficult to treat, partly because most people are very unwell by the time they are diagnosed. It is also rare, which makes it difficult to determine which treatment gives the best outcome. Lymphoma specialists (haematologists or oncologists) and bowel specialists (gastroenterologists) work together to plan the most appropriate treatment for each individual person with intestinal T-cell lymphoma.

Your specialist might ask you if you would like to take part in a clinical trial to help test what the best treatment is for intestinal T-cell lymphoma. If you don’t want to take part in a clinical trial, or if there isn’t one that is suitable for you, you are likely to be treated with chemotherapy. You might have surgery to remove the affected parts of your bowel first.

The most common chemotherapy regimens used for intestinal T-cell lymphoma are:

• CHOP: cyclophosphamide, doxorubicin (or hydroxydaunorubicin), vincristine (also known as Oncovin®) and prednisolone
• CHEOP: CHOP plus etoposide
• IVE/MTX: ifosfamide, etoposide (also known as Vepicid®) and epirubicin with methotrexate 

If you respond to chemotherapy and you are well enough, your medical team are likely to recommend that you have a self (autologous) stem cell transplant. This might give you a better chance of staying in remission (no evidence of lymphoma) after treatment.

You are also likely to need support to make sure you get enough nutrients. You might have a feeding tube so that you can be fed liquid food or you might be fed through a tube into a vein (intravenously). If you have coeliac disease, it is important to follow a gluten-free diet to help to avoid further damage to your small bowel. The Coeliac UK website has information about the gluten-free diet.

Extranodal NK/T-cell lymphoma, nasal type

Extranodal NK/T-cell lymphoma, nasal type, is a rare fast-growing (high-grade) non-Hodgkin lymphoma. It grows outside the lymphatic system (‘extranodal’), usually in the nose (‘nasal’). The cells of origin are thought to be two different kinds of lymphocyte (white blood cell):

• natural killer (NK) cells
• cytotoxic T cells.

Extranodal NK/T-cell lymphoma, nasal type, is more common in people from Asia, Central America and South America. It is very rare in the UK with only around 20 people diagnosed each year. It usually develops in people around 50 to 60 years old. It is more common in men than women.

Extranodal NK/T-cell lymphoma, nasal type, is strongly linked to infection with a virus called Epstein-Barr virus (EBV). EBV is a very common virus, that can cause glandular fever. After you’ve been infected with it, EBV stays in your body but your immune system normally keeps it under control. Most people who have EBV infection do not develop lymphoma. Scientists are investigating why some people who have been infected with EBV go on to develop extranodal NK/T-cell lymphoma.

People who have extranodal NK/T-cell lymphoma, nasal type, usually develop a fast-growing lump inside their nose or in the sinuses (air-filled spaces) around the nose. You might have symptoms that affect your nose, eyes or face, such as:

• a blocked nose
• nosebleeds, or nasal crusting
• swelling or pain in your face
• weepy eyes (blocked tear ducts) or eye discomfort.

The lymphoma might get bigger and grow into the roof of your mouth, or into your throat and eye sockets. 

It is also common to have general symptoms, such as:

• swollen lymph nodes
• fever, night sweats and weight loss (‘B symptoms’)
• fatigue.

If the lymphoma affects your skin, you might have a rash or raised, red lumps that can break down (ulcerate) and scab over.

For the other types of NKT cell lymphoma that develop outside of the nose, it can spread to your lymph nodes, skin, testicles or gut.

Treatment of extranodal NK/T-cell lymphoma, nasal type

Extranodal NK/T-cell lymphoma, nasal type, is rare. This makes it difficult to determine the most suitable treatment approach. Clinical trials in this type of lymphoma are unusual because it is such a rare condition.

For localised NK/T-cell lymphoma of the nose, radiotherapy is the most common treatment for early staged extranodal NK/T-cell lymphoma, nasal type. You often also have chemotherapy, either at the same time or after your radiotherapy.

If your lymphoma is more advanced (stage 3 or 4), you are likely to have chemotherapy without radiotherapy.

Chemotherapy used to treat extranodal NK/T-cell lymphoma, nasal type, includes regimens (combination of drugs) such as:

• DDGP: dexamethasone, cisplatin, gemcitabine and pegylated asparaginase
• SMILE: dexamethasone (a steroid), methotrexate, ifosfamide, L-asparaginase and etoposide
• GELOX: gemcitabine, L-asparaginase, oxaliplatin.

If you have advanced lymphoma, you respond to chemotherapy and you are well enough, your doctor might recommend that you have a self (autologous) or donor (allogenic) stem cell transplant. This might give you a better chance of staying in remission (no evidence of lymphoma).

Hepatosplenic T-cell lymphoma

Hepatosplenic T-cell lymphoma is a very rare type of fast-growing (high-grade) non-Hodgkin lymphoma that grows in the liver and spleen. 

‘Hepato’ means ‘relating to the liver’; ‘splenic’ means ‘relating to the spleen’.

Hepatosplenic T-cell lymphoma is a very rare condition. It usually affects younger adults, typically people in their mid-30s. It is more common in men than women.

Hepatosplenic T-cell lymphoma is linked with low function of the immune system. It is more likely to develop in people who are taking medicines that suppress the immune system – for example, after a transplant or for inflammatory bowel diseases such as Crohn’s disease. However, it can develop spontaneously in people who don’t have pre-existing conditions and aren’t taking medication.

The most common symptoms of hepatosplenic T-cell lymphoma are:

• a swollen liver, which might make you feel bloated, cause fluid to build-up in your tummy, or make your skin or the whites of your eyes look yellow (jaundice)
• a swollen spleen, which might cause pain behind your ribs on the left side, or make you feel full very quickly when you’re eating
• anaemia (low red blood cell count), which can make you tired and short of breath
• thrombocytopenia (low platelet level), which can make you bruise or bleed more easily than normal
• neutropenia (low neutrophil level), which makes you more prone to infections than usual
• fever, night sweats and weight loss (‘B symptoms’).

Unlike most other lymphomas, hepatosplenic T-cell lymphoma doesn’t usually cause swollen lymph nodes. It can be difficult to diagnose because the lymphoma cells are scattered around your body rather than in lumps.

Most people with hepatosplenic T-cell lymphoma have advanced stage lymphoma (stage 3 or 4) when they are diagnosed.

Treatment of hepatosplenic T-cell lymphoma

Hepatosplenic T-cell lymphoma is rare. This makes it difficult to determine the most suitable treatment approach. Clinical trials in this type of lymphoma are unusual because it is such a rare condition. However, some trials of new treatments do allow people with hepatosplenic T-cell lymphoma to take part, particularly if their lymphoma has relapsed. Your doctor might ask you if you would like to take part in a clinical trial to help find out what the best treatment is for hepatosplenic T-cell lymphoma.

If you don’t want to take part in a clinical trial, or if there isn’t one that is suitable for you, you are likely to be treated with chemotherapy. The most common chemotherapy regimens used for hepatosplenic T-cell lymphoma are:

• ICE: ifosfamide, carboplatin and etoposide
• IVAC: ifosfamide, etoposide (also known as VP-16) and cytarabine (also known as Ara-C)
• In certain situations CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine (also known as Oncovin®) and prednisolone) or CHEOP (CHOP plus etoposide) might be considered.

If you respond to chemotherapy and you are well enough, your doctor might recommend that you have a stem cell transplant. A stem cell transplant might give you a better chance of staying in remission (no evidence of lymphoma).

Back to top

Outlook of T-cell 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 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. 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 and some treatments will have improved since then. Those people may not have received the same treatment that you will receive. 

Back to top

Follow-up of T-cell lymphomas

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.

Back to top

Relapsed or refractory T-cell lymphomas

It is common for T-cell lymphoma to come back (relapse) after being in remission. Sometimes, T-cell lymphoma doesn’t respond to treatment (refractory lymphoma).

The treatment at this stage depends on:

• what treatment or treatments you’ve had before
• how well your previous treatment worked
• how long your remission lasted
• how your lymphoma is behaving now
• your age, general health and feelings about further treatment
• the treatments available (including clinical trials).

Treatment options often include:

• a different chemotherapy regimen
• a targeted drug, usually through a clinical trial
• a donor (allogeneic) stem cell transplant, if your lymphoma responds to chemotherapy and you are well enough
• radiotherapy to help treat any symptoms caused by large lumps of lymphoma in a particular area of your body.

Back to top

Research

Scientists are testing many different treatments for T-cell lymphomas, including some treatments that are already approved for other types of lymphoma. These include:

• antibody treatments  
• antibody–drug conjugates  
• immune checkpoint inhibitors  
• proteasome inhibitors
• HDAC inhibitors
• immunomodulatory drugs.

Some of these might be available to you through a clinical trial. If you are interested in taking part in a clinical trial, ask your doctor if there is a trial that might be suitable for you. To find out more about clinical trials or search for a trial that might be suitable for you, visit Lymphoma TrialsLink.

Back to top