Anaplastic large cell lymphoma

Anaplastic large cell lymphoma (ALCL) is a rare type of non-Hodgkin lymphoma (NHL). It is more common in children and young adults.

ALCL develops when white blood cells called T-cells become abnormal. These usually build up in the lymph nodes, but can affect other parts of the body.

Usually, the first symptom is a painless swelling in the neck, armpit or groin. General symptoms may include loss of appetite and tiredness. Some people may also have night sweats, high temperatures (fevers) or weight loss. Other symptoms depend on where the lymphoma is in the body.

The most common test for ALCL is to remove a sample of an enlarged lymph node and check it for lymphoma cells. You may also have other tests and scans to find out more about the lymphoma. Doctors describe ALCL as ALK-positive or ALK-negative. This depends on whether the lymphoma cells have a protein called anaplastic lymphoma kinase (ALK).

Most people are treated with chemotherapy and steroids. Some people may also have radiotherapy or stem cell treatments.

Your doctor may invite you to take part in a clinical trial if a new treatment is being researched for ALCL.

What is anaplastic large cell lymphoma (ALCL)?

It is best to read this information with our general information about non-Hodgkin lymphoma (NHL). If you have any more questions, you can ask your doctor or nurse at the hospital where you are having treatment.

Anaplastic large cell lymphoma (ALCL) is a rare type of fast-growing NHL. It develops when T-cells (also called T-lymphocytes) become abnormal. T-cells are white blood cells that fight infection.

The abnormal T-cells (lymphoma cells) usually build up in lymph nodes, but they can affect other parts of the body.

People with ALCL are divided into two groups. If the lymphoma cells have a protein called anaplastic lymphoma kinase (ALK), the lymphoma is described as ALK-positive. If the protein is not there, the lymphoma is described as ALK-negative. The treatment of the two sub-types of ALCL may be different. Your doctor or specialist nurse will explain more about this.

ALCL can affect all ages, but it is most common in children and young adults. We have more information for teenagers and young adults with cancer. We also have information about children’s cancers. For more information about lymphoma in children, contact the Children’s Cancer and Leukaemia Group.


Causes and risk factors for ALCL

The causes of ALCL are mostly unknown. Like other cancers, it is not infectious and cannot be passed on to other people. It is more likely to affect children and young adults, and it is more common in males than females.

Very rarely, breast implants are linked to ALCL. The lymphoma often only affects the area around the breast implant and is treated with surgery.

We have more information about risk factors for lymphoma.


Signs and symptoms of ALCL

Usually, the first sign of ALCL is a painless swelling in the neck, armpit or groin.

This 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.

ALCL 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.

B symptoms

Some people may also have:

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

These are called B symptoms.


Diagnosing 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.

Other tests may include:

  • blood tests
  • x-rays and scans
  • bone marrow samples.

Doctors use the information from all these tests to find out more about the lymphoma, such as its stage and grade.


Staging and grading ALCL

Staging

The stage of the lymphoma describes which areas of the body are affected by lymphoma. This information helps doctors plan the right treatment for you.

The stage of a lymphoma is usually described using numbers from 1 to 4. Stages 1 and 2 are also called early-stage, limited or localised lymphoma. Stages 3 and 4 are also called advanced lymphoma.

As well as giving each stage a number, doctors often add the letters A or B. A means you do not have B symptoms. B means you do have B symptoms.

Sometimes the lymphoma can affect areas outside the lymph nodes. This is called extranodal lymphoma, and the stage will include the letter E (for extranodal).

Grading

Non-Hodgkin lymphomas are divided into two groups:

  • Low-grade (indolent) lymphomas, which usually grow slowly.
  • High-grade (aggressive) lymphomas, which grow more quickly.

ALCL is a high-grade, fast-growing lymphoma.


Treating ALCL

Your treatment may depend on:

  • the stage of the lymphoma
  • your general health
  • whether the lymphoma is ALK-positive or ALK-negative.

Most people will have a combination of drugs that include chemotherapy and steroids. Some people may also have radiotherapy or stem cell treatments.

It is common for ALCL to be treated as part of a clinical trial. This is a way of researching newer drugs and combinations of drugs that may be effective for this type of lymphoma.

ALCL usually responds well to chemotherapy, and treatment may make it disappear (called remission). There’s a risk that the lymphoma may come back (relapse) in the future. If this happens, chemotherapy and stem cell transplants can be used to try to control it.

Chemotherapy

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

ALCL is usually treated with a combination of chemotherapy and other drugs.

The most common drug treatment is called CHOP. Other combinations of drugs may also be used. This is often as part of a clinical trial.

CHOP is named after the initials of the drugs used. It includes:

  • the chemotherapy drugs cyclophosphamide, doxorubicin (hydroxydaunomycin) and vincristine (Oncovin®)
  • a steroid called prednisolone.

The chemotherapy drugs are given into a vein (intravenously). You take the steroid as tablets. You usually have treatment every 2 or 3 weeks. You have up to 8 treatments over several months.

More intensive combinations of drugs may be used to treat young adults and children with ALCL. This may mean longer stays in hospital.

Steroids

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

Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to nearby healthy 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.

Stem cell treatments (transplants)

This treatment is sometimes used if there are still signs of ALCL or it comes back after other treatments. It is an intensive treatment, so it is not suitable for everyone.

Stem cells are a type of blood cell that can make all other types of blood cells. There are two different types of stem cell treatment:

High-dose treatment with stem cell support (autologous stem cell transplant)

Some people have treatment to put the lymphoma into remission. Then some of their own stem cells are collected from their blood and stored. Then they have high doses of chemotherapy to try to destroy any remaining lymphoma cells. After this, their stem cells are returned through a drip (like a blood transfusion). The stem cells help their blood cells recover from the effects of chemotherapy.

Donor transplant (allogeneic transplant)

Some people have chemotherapy to put the lymphoma into remission and are then given stem cells from another person (a donor).

Targeted therapy

Targeted therapies (also called biological therapies) are drugs that use unique features of the cancer to find and treat cancer cells. These drugs only ‘target’ the cancer cells, so they have less effect on healthy cells.

A drug called brentuximab vedotin is used if there are still signs of ALCL or it comes back after other treatments.

Other types of targeted therapy may be used to treat ALCL as part of a clinical trial.

Clinical trials

Your lymphoma doctor may talk to you about having treatment as part of a clinical trial. Clinical trials test new treatments or new ways of giving treatments.


Follow-up after treatment

After treatment, you will have regular check-ups. 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. We have more information about follow-up.


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. You can also call our cancer support specialists free on 0808 808 00 00. The organisations below also offer information and support:

  • Bloodwise offers support and information to people affected by blood cancers, including lymphoma.
  • 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.