Peripheral T cell lymphoma (PTCL)
Peripheral T cell lymphomas (PTCLs) are a group of fast growing non-Hodgkin lymphomas (NHLs).
NHL is a type of blood cancer that affects white blood cells called 
. It is a cancer of the .
There are many different types of PTCL. On this page we have information about:
- peripheral T cell lymphoma, not otherwise specified (PTCL-NOS)
- anaplastic large cell lymphoma (ALCL)
- angioimmunoblastic T cell lymphoma (AITL)
- EBV positive NK/T-cell lymphoma
- enteropathy associated T cell lymphoma (EATL)
- hepatosplenic gamma delta T cell lymphoma (HSTCL)
We have a separate page about T cell lymphoma of the skin. This is also called cutaneous T cell lymphoma (CTCL).
What are peripheral T cell lymphomas (PTCLs)?
Peripheral T cell lymphomas (PTCLs) are a rare type of non-Hodgkin lymphoma (NHL).
Lymphoma develops when white blood cells called lymphocytes grow out of control. They move around your body in your blood and your lymphatic system.
The lymphatic system is an important part of our . It has tubes that branch through all parts of the body.
These tubes are called lymph vessels or lymphatic vessels and they carry a straw coloured liquid called lymph. This liquid circulates around the body tissues. It contains a high number of white blood cells (lymphocytes) which fight infection.
There are different types of lymphocytes. The 2 main types are T lymphocytes (T cells) and B lymphocytes (B cells).
PTCL develops when the body makes abnormal T cells. So it is a type of T cell lymphoma. The abnormal lymphocytes build up in the or other body organs. They don't work properly. So they can't fight infection as normal white blood cells do.
It's fast growing and doctors call it a .
How common is peripheral T cell lymphoma (PTCL)?
This type of lymphoma is rare. Each year around 520 people are diagnosed with PTCL in the UK.
Symptoms of peripheral T cell lymphoma (PTCL)
Symptoms can start or get worse in just a few weeks. The symptoms can vary depending on what type of lymphoma you have, and where it is in your body.
Painless swellings
The most common symptom is one or more painless swellings in the:
- neck
- armpit
- groin
These swellings are enlarged . They can grow very quickly.
General symptoms (B symptoms)
You might have other general symptoms such as:
- heavy sweating at night
- high temperatures that come and go with no obvious cause
- losing a lot of weight
Doctors call this group of symptoms B symptoms. Some people may also have unexplained itching. It is important to tell your doctor about any symptoms like this.
Other symptoms
Lymphoma can grow in the bone marrow. If this happens, lymphoma cells can take up the space of normal blood cells. This can cause low blood cell counts. Symptoms of this include:
- tiredness and shortness of breath
- bleeding and bruising
You might have a swollen or liver. This can cause bloating and you might have tummy discomfort or pain.
The lymphoma cells make abnormal proteins and the body reacts to these. Doctors call this an autoimmune reaction. This can cause symptoms such as:
- skin rashes
- inflammation of your joints
What tests do you need to find out you have peripheral T cell lymphoma (PTCL)?
It can sometimes be hard to diagnose T cell lymphomas. It might take a long time and lots of tests before the doctors can make the diagnosis.
The main test to diagnose PTCL is a . A doctor removes a sample from the affected area. This often means removing part or all of a swollen lymph node. They send it to the laboratory for a specialist to look at it under a microscope.
You usually also have some blood tests.
If your doctor diagnoses PTCL, you usually have further tests. These might include:
- a
PET-CT scan - a
CT scan - a
bone marrow test to check if you have lymphoma cells in your bone marrow
Stages and grade of peripheral T cell lymphomas
Grade
Grade describes how the cells look under a microscope. Your grade tells the doctor how quickly the lymphoma is likely to grow and spread. Doctors put NHL into 2 grades:
- low grade (slow growing)
- high grade (fast growing)
PTCLs are fast growing and doctors call them high grade lymphomas.
Stage
You have tests to find out:
- where in your body you have lymphoma
- how many areas of lymphoma there are in your body
This is your stage. Doctors use your stage to plan your treatment.
There are different systems for staging NHL. Your doctor can tell you more about your stage, and what it means.
There are 4 main stages of NHL. These are stage 1 to stage 4. Or your doctors might talk about early (limited) stage and advanced stage lymphoma. Early stage usually means either stage 1 or stage 2 lymphoma. Advanced stage usually means either stage 3 or stage 4 lymphoma.
Types of peripheral T cell lymphoma (PTCL)
Peripheral T cell lymphomas (PTCL) are divided into subtypes. They all have very different characteristics and behaviour. Knowing which type you have will help your doctors recommend treatment.
T cell lymphomas are rare in the UK. So the different sub-types are all rare cancers.
Peripheral T cell lymphoma, not otherwise specified (PTCL NOS)
The most common subtype is peripheral T cell lymphoma, not otherwise specified (PTCL NOS). PTCL NOS includes all T cell lymphomas that don't fit into another group.
Most people with PTCL NOS have lymphoma cells only in the lymph nodes. But they can also develop in the liver, , and skin.
Anaplastic large cell lymphoma (ALCL)
This is a type of T cell lymphoma. The lymphoma cells look large and abnormal under a microscope. This is what the term anaplastic means.
There are different types of ALCL. Some ALCL cells have a genetic change (mutation). This means that they make ALK. ALK stands for a protein called anaplastic lymphoma kinase. Doctors test the lymphoma cells to look for ALK.
The different types of ALCL are:
- ALK positive ALCL
- ALK negative ALCL
- breast implant associated ALCL
- primary cutaneous (skin) ALCL
Angioimmunoblastic T cell lymphoma (AITL)
Angioimmunoblastic T cell lymphoma is a high grade (aggressive) lymphoma that affects T cells. Around 140 people are diagnosed with AITL in the UK every year.
It usually affects older people, typically around the age of 70. But you can develop it at any age.
EBV positive NK/T-cell lymphoma
This is a rare type of lymphoma. It can affect T cells or NK cells. NK stands for natural killer cells. NK cells are a type of lymphocyte, similar to B cells and T cells.
This type of lymphoma is linked to infection with the Epstein Barr virus (EBV). You can get it:
- inside your lymph nodes and lymphatic system – this is called EBV positive nodal NK/T cell lymphoma
- outside your lymphatic system, for example in your nose – this is called extranodal NK/T cell lymphoma
Enteropathy associated T cell lymphoma (EATL)
This is a very rare type of T cell lymphoma. It is also called enteropathy type T cell lymphoma (ETTL) or intestinal T cell lymphoma (ITCL).
EATL usually happens in the . It most often starts in the middle part (jejunum) or the lower part closest to the large intestine (ileum). It can spread to the liver, , lymph nodes, , stomach, or skin.
Hepatosplenic gamma delta T cell lymphoma (HSTCL)
This very rare type of T cell lymphoma starts in the liver or spleen. It tends to grow very quickly.
HSTCL can affect people with , who have a weak . It can also affect people who don't have any previous health problems.
Treatment for peripheral T cell lymphoma
Your treatment depends on:
- what type of PTCL you have
- where your lymphoma is (the stage)
- your symptoms
- your general health
Treatments include:
- a combination of chemotherapy drugs
- a stem cell transplant
- steroids
- targeted cancer drugs - you might have these as part of a clinical trial
- radiotherapy
- surgery - this is rare
Chemotherapy
Chemotherapy uses anti cancer (cytotoxic) drugs to destroy cancer cells. The drugs circulate throughout the body in your bloodstream. Chemotherapy is one of the main treatments for NHL.
There are many chemotherapy drugs. You usually have a several chemotherapy drugs together. The combination you have depends on what type of PTCL you have.
Targeted cancer drugs
Targeted cancer drugs work by targeting the differences in cancer cells that help them to grow and survive. There are many different types of targeted drugs.
Targeted cancer drugs are a common treatment for some types of NHL. But it isn't a common treatment for PTCL.
You might have a type of targeted cancer drug for ALK positive or ALK negative anaplastic large cell lymphoma (ALCL). You have chemotherapy together with the targeted drug called brentuximab vedotin.
Stem cell transplant
A stem cell transplant allows you to have very high doses of chemotherapy. The chemotherapy kills the lymphoma cells and also the stem cells in your . After the chemotherapy you have the new into your bloodstream through a drip.
You can have a transplant using:
- your own stem cells (autologous stem cell transplant)
- a donor’s stem cells (allogeneic stem cell transplant)
You usually have chemotherapy such as CHOP as your first treatment. The aim of this is to make your lymphoma go away so you are in .
If you are in remission, you might then have high doses of chemotherapy and a stem cell transplant. Doctors call this consolidation treatment. The aim of the transplant is to make the lymphoma stay away rather than to make the lymphoma go away in the first place.
Generally, doctors only recommend a stem cell transplant if you are already in a remission. This is intensive treatment so you also need to be well enough to have it.
it is most common to have an autologous stem cell transplant.
Radiotherapy
Radiotherapy uses high energy rays similar to x-rays to destroy cancer cells.
Your doctor might offer you radiotherapy as part of your treatment for some types of early stage PTCLs. In some situations, you have radiotherapy alongside chemotherapy. This is called chemoradiotherapy.
Surgery
Surgery to remove lymphoma isn't a common treatment. But you might have an operation to remove breast implant associated ALCL. You usually have surgery to remove the implant, and any lumps or fluid.
Treatment for the different types of PTCL
Your treatment depends on what type of PTCL you have.
You usually have a several chemotherapy drugs together. The combination you have depends on what type of PTCL you have.
A common combination of chemotherapy drugs is called CHOP. This is a mixture of chemotherapy and steroids. It includes the drugs cyclophosphamide, doxorubicin, vincristine and prednisolone. Sometimes you have the chemotherapy drug etoposide added to the treatment (CHOEP).
Your doctor might offer you radiotherapy as part of your treatment if your lymphoma is at an early stage.
If you are in remission, you might then have high doses of chemotherapy and a stem cell transplant. Doctors call this consolidation treatment. The aim of the transplant is to make the lymphoma stay away rather than to make the lymphoma go away in the first place.
This is intensive treatment so you need to be well enough to have it.
It is most common to have a stem cell transplant using your own stem cells. This is called an autologous stem cell transplant.
There are different types of ALCL including:
- ALK positive ALCL
- ALK negative ALCL
- breast implant associated ALCL
ALK positive and ALK negative ALCL
You might have chemotherapy together with the targeted drug called brentuximab vedotin. You often have the chemotherapy drugs cyclophosphamide, doxorubicin and the steroid prednisolone (CHP).
Your doctor might offer you radiotherapy as part of your treatment if your lymphoma is at an early stage.
You might then have a stem cell transplant after your chemotherapy if:
- the chemotherapy has worked well
- you have ALK negative ALCL
This is intensive treatment so you need to be well enough to have it.
It's most common to have a stem cell transplant using your own stem cells. This is called an autologous stem cell transplant.
Breast implant associated ALCL
You might not need chemotherapy for breast implant associated ALCL. You usually have surgery to remove the implant and any lumps or fluid. If you do need more treatment, you might have CHOP chemotherapy.
You usually have a several chemotherapy drugs together.
A common combination of chemotherapy drugs is called CHOP. This is a mixture of chemotherapy and steroids. It includes the drugs cyclophosphamide, doxorubicin, vincristine and prednisolone. Or you might have a similar treatment combination to this.
Your doctor might offer you radiotherapy as part of your treatment if your lymphoma is at an early stage.
If the chemotherapy has worked well, you might go on to have a stem cell transplant. This is intensive treatment so you need to be well enough to have it.
It's most common to have a stem cell transplant using your own stem cells. This is called an autologous stem cell transplant.
You usually have a combination of chemotherapy drugs. Possible treatment combinations include:
- DDGP – dexamethasone, cisplatin, gemcitabine and pegasparagase
- SMILE – dexamethasone, methotrexate, ifosfamide, L-aspariginase and etoposide
- GELOX – gemcitabine, L-asparaginase and oxaliplatin
Your doctor might offer you radiotherapy as part of your treatment for early stage extranodal NK/T cell lymphoma. You might have it together with chemotherapy. This is called chemoradiotherapy.
You might have a stem cell transplant after your chemotherapy, if you responded well to the chemotherapy. This is intensive treatment so you need to be well enough to have it.
It is most common to have a stem cell transplant using your own stem cells. This is called an autologous stem cell transplant.
You usually have a several chemotherapy drugs together.
A common combination of chemotherapy drugs is called CHOP. This is a mixture of chemotherapy and steroids. It includes the drugs cyclophosphamide, doxorubicin, vincristine and prednisolone. Your doctor might add other drugs to this regime.
If the chemotherapy has worked well, you might go on to have a stem cell transplant. This is intensive treatment so you need to be well enough to have it.
It is most common to have a stem cell transplant using your own stem cells. This is called an autologous stem cell transplant.
There is no for this rare sub-type of lymphoma. This means treatment might vary, depending on what chemotherapy drug your doctors recommends.
You usually have a combination of chemotherapy drugs. This might be a more intensive regime than CHOP which is the treatment for other types of NHL. You might have:
- IVAC – ifosfamide, etoposide and cytarabine
- ICE – ifosfamide, carboplatin and etoposide
If the chemotherapy works well, you might go on to have a stem cell transplant after your chemotherapy. This is intensive treatment so you need to be well enough to have it.
It is most common to have a stem cell transplant using your donor stem cells. This is called an allogeneic stem cell transplant.
Lymphoma that does not go away with treatment is called refractory lymphoma. Lymphoma that comes back after treatment is called relapsed lymphoma. You might have:
- further chemotherapy treatment either using either the same or different chemotherapy drugs
- a stem cell transplant using either your own stem cells or donor stem cells
- targeted cancer drugs
Where possible, your team will consider offering you treatments as part of a clinical trial.
Research into peripheral T cell lymphoma (PTCL)
Researchers around the world are looking at the treatments for NHL. Your doctor might suggest you take part in a .
Survival for peripheral T cell lymphoma
The best person to talk to you about your outlook or prognosis is your doctor or nurse specialist. Not everyone wants to know. People cope differently with their lymphoma and want different information.
Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.
Coping with NHL
It can be difficult finding our you have NHL. Some people find it can be particularly worrying in the run up to check up appointments.
Advice and support is available to help you cope with NHL and its treatment.
Cancer Chat is an online forum where you can get support. It is for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.
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