Peripheral T-cell lymphoma

What is peripheral T-cell lymphoma (PTCL-NOS)?

T-cell lymphomas are a type of non-Hodgkin lymphoma (NHL). They develop when T-cells (also called T-lymphocytes) become abnormal. T-cells are white blood cells that fight infection.

There are different types of T-cell lymphoma. The most common group is peripheral T-cell lymphoma (not otherwise specified). It is also called PTCL-NOS. The lymphomas in this group are not all the same. But they develop in a similar way and are treated in the same way.

PTCL-NOS is a fast-growing lymphoma. The abnormal T-cells (lymphoma cells) usually build up in lymph nodes, but can affect other parts of the body.

Symptoms of peripheral T-cell lymphoma (PTCL-NOS)

Painless swelling in neck, armpit or groin

This is the most common sign of PTCL-NOS. It is caused by lymphoma cells building up in the lymph nodes, which makes them bigger. Often, lymph nodes in more than one part of the body are affected.

B symptoms

Some people also have:

drenching night sweats
high temperatures (fevers) with no obvious cause
unexplained weight loss

Doctors call this group of symptoms B symptoms.

Other symptoms

PTCL-NOS may also affect other parts of the body outside the lymph nodes. This is called extranodal disease. Symptoms depend on the area affected.

General symptoms may include loss of appetite and tiredness.

Causes of peripheral T-cell lymphoma (PTCL-NOS)

The causes of lymphoma are mostly unknown. Like other cancers, it is not infectious and can not be passed on to other people.

We have more information about causes and risk factors of non-Hodgkin lymphoma.

Diagnosis of peripheral T-cell lymphoma (PTCL-NOS)

Biopsy for lymphoma

The most common test for this lymphoma is to remove part or all of an enlarged lymph node (a biopsy). This may be done under local or general anaesthetic. The biopsy is then sent to a laboratory to be checked for lymphoma cells. You may also have biopsies taken from other areas of the body.

You can read more about further tests you may have in our information about non-Hodgkin lymphoma.

Waiting for test results can be a difficult time. It may help to talk to your family, friends or specialist nurse.

Staging and grading of peripheral T-cell lymphoma (PTCL-NOS)

The results of your tests help your doctors find out how many areas of the body are affected by lymphoma and where these areas are. This is called staging.

Lymphomas are also grouped as either low-grade or high-grade. Low-grade lymphomas are usually slow growing and high-grade lymphomas grow more quickly. PTCL-NOS is a high-grade, fast-growing lymphoma.

We have more information about staging and grading of non-Hodgkin lymphoma.

Treatment for peripheral T-cell lymphoma (PTCL-NOS)

A team of specialists will meet to discuss the best possible treatment for you. This is called a multidisciplinary team (MDT).

Your doctor or cancer specialist or nurse will explain the different treatments and their side effects. They will also talk to you about things to consider when making treatment decisions.

Your treatment may depend on the stage of the lymphoma and your general health.

Most people will have a combination of drugs that include chemotherapy and steroids. Some people will then have stem cell treatment. Sometimes radiotherapy may be used after chemotherapy to treat an area of the body affected by lymphoma.

Treatments may make all signs of the PTCL-NOS disappear (called remission). But PTCL-NOS may come back (called recurrence or relapse). If this happens, further treatment can be given.

Treatments for PTCL-NOS may include:

Chemotherapy

PTCL-NOS is usually treated with a combination of chemotherapy and other drugs. The most commonly used drugs are in a treatment called CHOP.
Steroids

Steroids are drugs that are often given with chemotherapy to treat lymphomas. They help make chemotherapy more effective.
Stem cell treatment (transplants)

If chemotherapy has treated the PTCL-NOS effectively, some people will then have stem cell treatment. This may also be used to treat PTCL-NOS that has come back. It is an intensive treatment, so it is not suitable for everyone. You may have a transplant using your own stem cells (autologous stem cell transplant) or cells from a donor (allogeneic stem cell transplant).
Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to nearby healthy normal cells cells. Radiotherapy only treats the area of the body that the rays are aimed at.

Some people have radiotherapy after chemotherapy to treat an area of lymphoma. This can treat any remaining lymphoma cells in the area. It can also reduce the risk of lymphoma coming back in the area. Radiotherapy is also sometimes given to treat symptoms such as pain.
Targeted therapy

Targeted therapies interfere with the way cancer cells grow. These drugs only ‘target’ the cancer cells, so they have less effect on healthy cells.

You may have some treatments as part of a clinical trial.

After peripheral T-cell lymphoma (PTCL-NOS) treatment

You have regular follow-up appointments after treatment. These appointments are a good opportunity for you to talk to your doctor or nurse about any concerns you have. Your doctor will want to know how you are feeling generally, and to check you are recovering from any side effects of treatment.

Late effects

Sometimes a side effect may continue or develop months or years after treatment. This is called a late effect.

We have more information about long-term and late effects of treatment for lymphoma.

Well-being and recovery

Even if you already have a healthy lifestyle, you may choose to make some positive lifestyle changes after treatment.

Making small changes such as eating well and keeping active can improve your health and wellbeing and help your body recover.

Getting support

Everyone has their own way of dealing with illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse.

Macmillan is also here to support you. If you would like to talk, you can:

Call the Macmillan Support Line on 0808 808 00 00.
Chat to our specialists online.
Visit our T-cell lymphomas forum to talk with people who have been affected by T-cell lymphoma, share your experience, and ask an expert your questions.

The organisations below also offer information and support:

Blood Cancer UK

Blood Cancer UK is a blood cancer research charity that provides information and support on any type of blood cancer.
Lymphoma Action

Lymphoma Action gives emotional support, advice and information on all aspects of Hodgkin lymphoma and non-Hodgkin lymphoma. It has a national network of people with lymphoma, and local groups.

About our information

References

Below is a sample of the sources used in our peripheral T-cell lymphoma (PTCL-NOS) information. If you would like more information about the sources we use, please contact us at cancerinformationteam@macmillan.org.uk

Schmitz M and de Leval L. How I manage peripheral T-cell lymphoma, not otherwise specified and angioimmunoblastic T-cell lymphoma: current practice and a glimpse into the future British Journal of Haematology. 2017. 176, 851–866.

Reviewers

This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by Senior Medical Editor, Dr Anne Parker, Consultant Haematologist.

Our cancer information has been awarded the PIF TICK. Created by the Patient Information Forum, this quality mark shows we meet PIF’s 10 criteria for trustworthy health information.