Anaplastic large cell lymphoma

Anaplastic large cell lymphoma (ALCL) is a rare type of non-Hodgkin lymphoma (NHL). Like all lymphomas, it’s a cancer of the lymphatic system, which is part of the body’s immune system.

ALCL is more common in children and young adults. It develops when white blood cells called T-cell lymphocytes divide in an uncontrolled way. These build up in parts of the body like the lymph nodes, lungs or skin. Some people have symptoms relating to where the lymphoma is in their body. For example, if the lymphoma is in the chest area, it may cause a cough or breathlessness.

General symptoms may include loss of appetite and tiredness. Some people may also have night sweats, high temperatures (fevers) or weight loss. These are known as B-symptoms.

Doctors describe ALCL as ALK-positive or ALK-negative. This depends on whether the lymphoma cells have a protein called anaplastic lymphoma kinase (ALK).

ALCL grows quickly and usually needs treatment straight away with chemotherapy. Some people may also have radiotherapy or stem cell transplants. Your doctor may invite you to take part in a clinical trial if a new treatment is being researched for ALCL.

ALCL often responds well to chemotherapy and disappears with treatment (remission). But sometimes it can come back and needs further treatment to try to control it.

Anaplastic large cell lymphoma (ALCL)

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma (NHL).

This information should be read with our general information about NHL. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having treatment.


Non-Hodgkin lymphoma (NHL)

Non-Hodgkin lymphoma (NHL) is a cancer of the lymphatic system. This system is part of the body’s immune system, which helps us fight infection.

It is made up of organs such as the bone marrow, thymus, spleen and lymph nodes (lymph glands).

The lymphatic system
The lymphatic system

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

Lymph fluid contains lymphocytes. These are white blood cells that help the body fight infection and disease.

Lymphocytes grow in the bone marrow, where blood cells are made. The two main types of lymphocyte are B-cells and T-cells. B-cells mature in the bone marrow. T-cells mature in the thymus gland behind the breastbone. When they are mature, both B-cells and T-cells help fight infections.

Lymphoma is a disease where either T-cells or B-cells grow in an uncontrolled way. There are different types of lymphoma. They are grouped depending on whether B-cells or T-cells are involved.


What is ALCL?

ALCL is a rare type of non-Hodgkin lymphoma that affects the T-cells. It is more likely to affect children and young adults, and is more common in males.

The abnormal T-cells build up in lymph nodes. They may also occur in other parts of the body, such as the skin or lungs.

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 isn’t there, the lymphoma is described as ALK-negative. The treatment of the two sub-types of ALCL may be slightly different. Your doctor or specialist nurse will explain more about this.

We have other information about NHL in children and NHL in young adults.


Causes of ALCL

The causes of ALCL are unknown.

Like other cancers, it’s not infectious and can’t be passed on to other people.


Signs and symptoms of ALCL

The first sign of ALCL is often a painless swelling in the neck, armpit or groin. This is caused by enlarged lymph nodes. Sometimes, lymph nodes in more than one part of the body are affected.

Some people have symptoms relating to where the lymphoma is in their body. For example, if the lymphoma is in the chest area, it may cause a cough or breathlessness.

General symptoms may include loss of appetite and tiredness.

Some people may also have night sweats, high temperatures (fevers) or weight loss. These are known as B-symptoms.


How ALCL is diagnosed

If you have an enlarged lymph node, the doctors will remove it during a small operation. This is called a biopsy. It may be done under a local or general anaesthetic. The lymph node will be examined under a microscope. The doctors may also take biopsies from other areas of the body.

You may have some other tests, such as:

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

These tests can find out more about the lymphoma, such as its stage and grade (see below). Doctors use this information to decide which treatment is best for you. We have more information about tests for non-Hodgkin lymphoma.


Staging and grading of ALCL

Staging

Your doctors will look at how many groups of lymph nodes are affected, and whether the lymphoma has spread to other organs. This is called staging.

A group of lymph nodes refers to lymph nodes in one area of the body, such as:

  • in the armpit
  • on one side of the neck
  • in the groin.

Stage 1

One group of lymph nodes in one area of the body is affected.

Stage 2

Two or more groups of lymph nodes are affected. All the affected nodes are either above the diaphragm or below the diaphragm. The diaphragm is a sheet of muscle under the lungs.

Stage 3

The lymphoma is in lymph nodes both above and below the diaphragm.

Stage 4

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

B-symptoms

Doctors add either the letter A or B after the stage number. This is to show whether you have any B-symptoms, such as a high temperature, night sweats or weight loss.

If you don’t have any of these symptoms, they add the letter A next to the stage. If you do have them, they add the letter B.

Sometimes, the lymphoma can start outside the lymph nodes. This is called extranodal lymphoma. In this case, the stage will include the letter E (for extranodal).

Grading

Doctors also divide non-Hodgkin lymphomas into two groups, depending on how they grow:

  • Indolent (low-grade) lymphomas grow slowly
  • Aggressive (high-grade) lymphomas grow quickly.

ALCL is an aggressive lymphoma. It is fast-growing and usually needs treatment straight away with chemotherapy.


Treating ALCL

Chemotherapy is the main treatment for ALCL. Some people may also have radiotherapy or stem cell treatments.

ALCL usually responds well to chemotherapy and treatment may make it disappear (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 drugs. The drugs are given into a vein (intravenously).

A chemotherapy treatment called CHOP is often used. You can have this treatment as an outpatient.

CHOP is named after the initials of the drugs used, which are:

cyclophosphamide

doxorubicin (hydroxydaunomycin)

vincristine (Oncovin®)

prednisolone (a steroid).

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

Steroid therapy

Steroids are often given with chemotherapy to treat lymphomas. 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 normal cells.

Radiotherapy may be given after chemotherapy. Occasionally, radiotherapy may be used alone if the lymphoma is stage 1 (see above).

Stem cell treatment (transplant)

Some people may have treatment using stem cells. These treatments are not suitable for everyone.

Stem cells are blood cells at their earliest stage of development. All blood cells develop from stem cells. There are two different types of stem cell treatment:

High-dose chemotherapy with stem cell support – People who have this type of stem cell treatment have some of their own stem cells collected and stored. This allows them to have higher doses of chemotherapy to destroy the lymphoma cells. After the chemotherapy, their stem cells are returned to them by a drip into a vein. This helps their blood cells recover from the effects of chemotherapy.

Donor (allogeneic) stem cell transplant – Some people may have treatment using stem cells from another person (a donor).

Targeted therapy

You may have a type of targeted therapy called a monoclonal antibody. Monoclonal antibodies such as brentuximab vedotin (Adcetris®) target certain proteins on the surface of cancer cells. Once brentuximab targets the protein, it releases a chemotherapy drug called vedotin into the cells.

Brentuximab vedotin may be used if ALCL does not respond to treatment or comes back after treatment. It may only be available in some situations. Your cancer doctor can tell you if it’s appropriate for you. Some people may be given it as part of a clinical trial.

If a drug isn’t available on the NHS, there may be different ways you are still able to have it. Your cancer doctor can give you advice. We have more information on what you can do if a treatment isn’t available.


Clinical trials

New treatments for ALCL are being researched all the time. Your doctor may invite you to take part in a clinical trial. Trials compare new treatments with the best available standard treatments. Before you enter a trial, your doctor must discuss the treatment with you and get your consent (permission).

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

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


Information and support

Everyone has their own way of dealing with their illness and the different emotions they experience. You may find it helpful to talk to family and friends or your doctor or nurse. You can also contact our cancer support specialists or the organisations listed below for more information and support.


Other useful organisations

Leukaemia CARE

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

Gives emotional support, advice and information on all aspects of Hodgkin lymphoma and non-Hodgkin lymphoma. Has a national network of people with lymphoma and local groups.