Peripheral T-cell lymphoma

Peripheral T-cell lymphoma (not otherwise specified – PTCL (NOS)) is a type of non-Hodgkin lymphoma. It develops when white blood cells called T-lymphocytes become abnormal – the lymphoma cells.

The lymphoma cells build up in lymph nodes and sometimes in other parts of the body such as the bone marrow, liver or skin. The most common symptom is a painless swelling in the neck, armpit or groin. Other symptoms may include: tiredness, weight loss, night sweats and high temperatures (fevers).

To diagnose DLBCL a doctor removes an enlarged lymph node and checks it for lymphoma cells. You will also have tests and scans to find out how many groups of lymph nodes are affected and whether the lymphoma has spread elsewhere. This is called staging.

PTCL (NOS) is fast-growing and needs to be treated quickly. This is usually with a combination of chemotherapy drugs. If a new treatment for PTCL (NOS) is being tested your doctor may invite you to take part in a clinical trial.

Peripheral T-cell lymphoma, not otherwise specified

This information is about a type of non-Hodgkin lymphoma known as peripheral T-cell lymphoma, not otherwise specified. It should ideally be read with our general information about non-Hodgkin lymphoma.

Non-Hodgkin lymphoma

Non-Hodgkin lymphoma (NHL) is a cancer of the lymphatic system. The lymphatic system is part of the body’s immune system and helps us fight infection. It’s made up of organs such as the bone marrow, thymus, spleen, and lymph nodes (or lymph glands). There is also lymphatic tissue in other organs such as the lungs, stomach and skin.

There are lymph nodes all over the body. They are connected by a network of tiny lymphatic vessels that contain a fluid called lymph. As lymph fluid flows through the lymph nodes, the nodes collect and filter out anything that the body doesn’t need, or that could harm the body. This includes bacteria, viruses, damaged cells, and cancer cells.

Lymph contains cells called lymphocytes. Lymphocytes are a type of white blood cell and are an essential part of the body’s defence against infection and disease.

Most lymphocytes start growing in the bone marrow, which is where blood cells are made. The two main types of lymphocytes are B-cells and T-cells. The B-cells continue to develop in the bone marrow. The T-cells go from the bone marrow to the thymus gland (behind the breast bone) and mature there. When they are mature, both B-cells and T-cells help us fight infections.

Lymphoma is a disease in which either T- or B-cells grow in an uncontrolled way.

There are many different types of non-Hodgkin lymphoma. They are grouped (or classified) according to certain characteristics. The most widely used classification system is produced by the World Health Organisation (WHO).

Peripheral T-cell lymphomas

Peripheral T-cell lymphomas (PTCL) are a type of NHL that make up less than 10% (1 in 10) of all cases of NHL in adults. There are different sub-types of PTCL depending on the characteristics of the T-cell.

The different sub-groups include:

  • peripheral T-cell lymphoma, not otherwise specified
  • anaplastic large cell lymphoma
  • angioimmunoblastic T-cell lymphoma
  • enteropathy-associated T-cell lymphoma
  • some very rare types of PTCL.

We have more detailed information about anaplastic large cell lymphoma. For other rare types of lymphoma, contact our cancer support specialists. They can give you more information about your illness and its treatment.

Peripheral T-cell lymphoma, not otherwise specified

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is a sub-type that is made up of all the peripheral T-cell lymphomas that don’t fit into any other sub-type and are therefore ‘not otherwise specified’ or ‘unspecified’. As scientists and doctors discover more about the different types of PTCL within this sub-group, it’s likely that further sub-groups will be identified.

PTCL, NOS can occur at any age but it’s more common in adults. It affects both men and women equally.


Causes

The causes of PTCL, NOS are unknown.

It is not infectious and can’t be passed on to other people.


Signs and symptoms

The first signs of lymphoma vary, but it’s often a painless swelling in the neck, armpit, or groin, caused by enlarged lymph nodes. Lymph nodes in several different areas are frequently involved. Some people experience a loss of appetite and tiredness.

A lymphoma can also affect other organs in the body such as the bone marrow, liver, spleen, stomach, bowel or the skin. It’s fairly common for PTCL, NOS to affect the bone marrow, liver and skin.

Other symptoms of lymphoma may include night sweats, unexplained high temperatures and weight loss. These are known as B symptoms and are fairly common with PTCL, NOS.


How it is diagnosed

The doctors at the hospital will remove an enlarged lymph node or part of it, and examine the cells under a microscope. This is called a biopsy. It’s a very small operation and may be done under local or general anaesthetic. You may also have biopsies taken from other parts of the body.

Further tests - including blood tests, x-rays, scans, and bone-marrow samples - are then used to get more information about the type of lymphoma and how far it has spread in the body. This information is used to help decide which treatment is most appropriate for you


Staging and grading

Staging

The stage of NHL describes how many groups of lymph nodes are affected, where they are in the body, and whether other organs such as the bone marrow or liver are affected.

Stage 1

The lymphoma is only in one group of lymph nodes, in one particular area of the body.

Stage 2

More than one group of lymph nodes is affected, but all the affected nodes are in either the upper half or the lower half of the body. The upper half of the body is above the diaphragm (the sheet of muscle underneath the lungs), and the lower half is below it.

Stage 3

Lymphoma is present in lymph nodes in both the upper and the lower parts of the body (ie in lymph nodes both above and below the diaphragm). The spleen is considered as a lymph node in this staging system.

Stage 4

The lymphoma has spread beyond the lymph nodes to other lymphatic organs, for example to sites such as the bone marrow, liver or lungs.

The stage usually includes the letter A or B, which describes whether B symptoms are present or not (eg stage 2B). Sometimes a lymphoma can start in areas outside the lymph nodes, and this is represented by the letter E, which stands for extranodal (eg stage 3AE).

Grading

For practical purposes, non-Hodgkin lymphomas are also divided into two groups, low- and high-grade. Low-grade lymphomas are usually slow-growing, and high-grade lymphomas tend to grow more quickly.

Peripheral T-cell lymphoma behaves as a high-grade lymphoma and usually needs prompt treatment.


Treatment

Chemotherapy is the main treatment for PTCL, NOS. Some people may also have stem cell treatments (see below). Radiotherapy is occasionally given after chemotherapy to treat early-stage disease (stages 1 and 2), or sometimes to help control symptoms (palliative radiotherapy).

PTCL, NOS can be quite difficult to treat successfully. Some people are cured by chemotherapy, but usually the chemotherapy will reduce and control the condition for a period of time (remission).

The risk of the PTCL, NOS coming back after initial treatment is quite high. If this happens, further chemotherapy can sometimes be given to try to control it again, but this isn’t always possible.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells.

A combination of chemotherapy drugs is usually used. Different combinations can be used to treat PTCL, and the best combination isn’t known. You may be asked to take part in a clinical trial that’s trying to find out which is the best to use.

The most commonly used chemotherapy combination is known as CHOP. It includes the chemotherapy drugs vincristine, doxorubicin, cyclophosphamide and the steroid prednisolone.

If the lymphoma doesn’t respond to CHOP or comes back at a later date, other chemotherapy combinations may be used.

High-dose treatment with stem cell support

High-dose treatment with stem cell support (autologous transplant) is sometimes used for this type of lymphoma. It may be offered to some people following their initial course of chemotherapy. It can sometimes be used if the lymphoma comes back, where it is again used after chemotherapy has been given.

High-dose treatment with stem cell support involves having intensive chemotherapy, which destroys the lymphoma cells but also destroys the blood cells in your bone marrow. Before you have the chemotherapy, you'll have some of your own stem cells (early blood cells) taken and stored. These are given back to you (like a blood transfusion) after the chemotherapy.

The stem cells make their way back to your bone marrow and start to produce new blood cells.

Your doctor will take into account your age and general health before recommending this treatment. This is because the intensity of treatment increases the risks of serious side effects.

Some people with PTCL, NOS may have treatment using stem cells from another person (a donor). This is called an allogeneic transplant and is mainly used if the lymphoma comes back (relapses) after initial treatment.

Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to healthy cells. It‘s sometimes used after chemotherapy for people whose lymphoma cells are contained in one area of the body (stage 1 or 2). Radiotherapy is also sometimes used to control symptoms (palliative radiotherapy).

Steroid therapy

Steroids are drugs that are often given with chemotherapy to help treat lymphomas. They also help you feel better and can reduce feelings of sickness (nausea).


Follow-up

After your treatment has been completed, your doctor will ask you to go back to hospital for regular check-ups. You will usually have a scan at the end of treatment to assess how well the treatment has worked. These scans aren’t usually carried out if you feel well. Your follow-up appointments are good opportunities to discuss with your doctor any worries or problems you may have. However, if you notice any new symptoms or are anxious about anything else between appointments, contact your doctor or nurse for advice.


Clinical trials

New treatments for PTCL, NOS are being researched all the time. You may be invited by your doctor to take part in a clinical trial to compare a new treatment with the best available standard treatment. Your doctor must discuss the treatment with you, and have your consent before entering you into any clinical trial.

Before any trial is allowed to take place, it must be approved by a research ethics committee, which protects the interests of those taking part.

You may decide not to take part or to withdraw from the trial at any stage. You will then receive the best standard treatment available.

Newer treatments for PTCL, NOS that are being researched in clinical trials include the chemotherapy drug gemcitabine and the biological therapy alemtuzumab.


Other useful organisations

The Lymphoma Association

The Lymphoma Association 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.

Leukaemia CARE

Leukaemia CARE is a national group promoting the welfare of people with leukaemia and other blood disorders, including non-Hodgkin lymphoma. Has regional support groups in many counties.

The Lymphoma Association

Provides information and emotional support to people with lymphoma and their families, carers and friends.