What are peripheral T-cell lymphomas, not otherwise specified?

Peripheral T-cell lymphomas (PTCLs) are a group of non-Hodgkin lymphoma (NHL). PTCLs develop when T-cells become abnormal (cancerous). T-cells are white blood cells that normally help fight infection. They are sometimes called T-lymphocytes.

There are different types of PTCL. Each type has different characteristics and behaves in a different way.

Any PTCL that does not fit into one of the types is grouped with others similar to it and called a peripheral T-cell lymphoma, not otherwise specified (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 are fast growing. The abnormal T-cells (lymphoma cells) usually build up in lymph nodes, but they can affect other parts of the body.

Symptoms of peripheral T-cell lymphoma, not otherwise specified

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 symptoms that doctors call B symptoms. These can include:

  • drenching night sweats which require a change of nightwear and bed covers
  • high temperatures (fevers) with no obvious cause
  • unexplained weight loss.

Knowing if you have any B symptoms will help your doctor to stage the lymphoma and plan your treatment.

Other symptoms

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

General symptoms of PTCL-NOS may include loss of appetite and tiredness.

Causes of peripheral T-cell lymphomas, not otherwise specified

The causes of peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS), are mostly unknown.  PTCL-NOS mainly affects adults in their 60s. They are more common in men than women.

Like other cancers, PTCL-NOS is not infectious. It cannot 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, not otherwise specified

The most common test for diagnosing lymphoma is a biopsy. A doctor will take a sample of tissue from the affected area. The most common place to take a biopsy from is an enlarged lymph node. This is called a lymph node biopsy. You may have all or a part of the lymph node removed. The tissue will be sent to a laboratory for testing. You may also have biopsies taken from other areas of your body.

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

Waiting for test results can be difficult. You may find it helpful to talk to your family, friends or specialist nurse.

The stages and grades of peripheral T-cell lymphomas, not otherwise specified

Your test results will help your doctors find out how many areas of your 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. High-grade lymphomas usually grow more quickly. Peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS) are high-grade lymphomas and normally develop quickly.

We have more information about the stages and grades of non-Hodgkin lymphoma.

Treatment for peripheral T-cell lymphoma, not otherwise specified

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

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

Treatment for peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS) depends on the stage of the lymphoma and whether you have symptoms that are causing problems.  

The most common treatments for PTCL-NOS are:

  • Chemotherapy

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

  • Steroids

    Steroids are drugs that are often given with chemotherapy to treat lymphomas. They help make chemotherapy more effective.

  • Stem cell 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.

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

We have more information about treating lymphoma that has come back.

After treatment for peripheral T-cell lymphoma, not otherwise specified

You will have regular follow-up appointments after your treatment. You can talk to your doctor or nurse about any concerns you may have at these appointments. Your doctor will want to know how you are feeling, and to check you are recovering from any side effects of treatment.

Late effects

Sometimes side effects may continue or develop months or years after treatment. These are called late effects. We have more information about long-term and late effects of treatment for lymphoma.

Sex life and fertility

Cancer and its treatment can sometimes affect your sex life. There ways to improve your sexual well-being and to manage any problems.

Treatment for lymphoma may affect your fertility. If you are worried about your fertility it is important to talk with your doctor before you start treatment. We have more information about:

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:

The organisations below also offer information and support:

  • Blood Cancer UK

    Blood Cancer UK offers support and information to people affected by blood cancers, including lymphoma.

  • Lymphoma Action

    Lymphoma Action gives emotional support, advice and information for people with Hodgkin lymphoma or non-Hodgkin lymphoma and those close to them. It has a national network of people with lymphoma, as well as local groups. Their website has a section called trialslink where you can see information about lymphoma clinical trials.

About our information

  • References

    Below is a sample of the sources used in our peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) information below. If you would like more information about the sources we use, please contact us at cancerinformationteam@macmillan.org.uk

    National Institute of Health and Care Excellence (NICE). Blood and bone marrow cancers. NICE Pathways. Last accessed 3 December 2020.

    d’Amore. F, Gaulard. P et al. Peripheral T-cell lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Volume 26, supplement 5, V108-V115. Published 01st September 2015. Available here: Peripheral T-cell lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up - Annals of Oncology

    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.

Reviewed: 01 March 2021
Reviewed: 01/03/2021
Next review: 01 March 2024
Next review: 01/03/2024