Types and grades
Non-Hodgkin lymphoma (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).
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 .
Find out more about how doctors group NHL
This type of lymphoma is rare. Each year around 520 people are diagnosed with PTCL in the UK.
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.
The most common symptom is one or more painless swellings in the:
neck
armpit
groin
These swellings are enlarged . They can grow very quickly.
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.
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
Read more general information about the symptoms of NHL
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
a
a to check if you have lymphoma cells in your bone marrow
Read more about tests to diagnose NHL
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.
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.
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.
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.
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 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.
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
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.
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.
Read more about angioimmunoblastic 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 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 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.
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 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 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.
Your treatment depends on what type of PTCL you have.
Researchers around the world are looking at the treatments for NHL. Your doctor might suggest you take part in a .
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.
More information about survival for 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.
Read more about coping with NHL
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.
Last reviewed: 20 Sept 2024
Next review due: 20 Sept 2027
Non-Hodgkin lymphoma (NHL) is a type of blood cancer that affects white blood cells called lymphocytes. It's the 7th most common type of cancer in adults.
There are many different types of non-Hodgkin lymphoma (NHL). Doctors also give NHL a grade, depending on how quickly it is likely to grow.
The stage tells you about the number and places in your body that are affected by lymphoma. There are 4 stages for NHL. Or doctors describe it as either early or advanced stage.
Your treatment depends on the type and stage of your NHL. Common treatments include chemotherapy, targeted and immunotherapy drugs, radiotherapy and a stem cell transplant.
Practical and emotional support is available to help you cope with non-Hodgkin lymphoma.
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