Anaplastic large cell lymphoma

This information page is about anaplastic large-cell lymphoma (ALCL). ALCL can be cutaneous (found only in the skin) or systemic (affecting the whole body). We have separate information on skin lymphomas. This information page is about systemic ALCL.

On this page

What is ALCL and who gets it?

Symptoms

Treatment

Relapsed and refractory ALCL

Research and targeted treatments

Follow-up

What is ALCL and who gets it?

Anaplastic large cell lymphoma (ALCL) is a type of mature T-cell lymphoma. Our overview page explains more about T-cell lymphoma.

The cancerous cells in ALCL are large and ‘anaplastic’, which means they look simpler and less specialised than normal T-cells.

Systemic ALCL accounts for about 1 in every 4 cases of T-cell lymphoma. Around 400 people are diagnosed with ALCL every year in the UK.

ALCL is divided into 2 types depending on whether or not a certain protein called ‘anaplastic large-cell kinase (ALK)’ is present in the cancerous T-cells. Only cells with a certain mutation (change) in their genes have ALK present. These are known as ‘ALK-positive ALCL’. It is not known what causes the mutation. Cases of ALCL that don’t have ALK are called ‘ALK-negative ALCL’.

The 2 types of ALCL usually affect different groups of people:

  • ALK-positive ALCL often affects people under 35. It is more common in males.
  • ALK-negative ALCL often affects older adults aged 40 to 65. It is slightly more common in men.

You might have different treatment depending what type of ALCL you have. In general, ALK-positive ALCL affects younger people and has a better outcome than ALK-negative ALCL.

If you are under 25 and have been diagnosed with ALCL, or if you have a child who has been diagnosed with ALCL, you might find our information on lymphoma in children and young people useful.

ALCL associated with breast implants

ALCL can be associated with breast implants, but this is extremely rare. This type of ALCL does not usually spread. It is often limited to the fluid or capsule that forms around the breast implant. If this is the case, you have the implant and the lymphoma removed by surgery and may not need any further treatment. You are then followed-up carefully to make sure all the lymphoma has been removed. If the lymphoma is more widespread, you need chemotherapy.

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Symptoms

Most people with ALCL are diagnosed at an advanced stage (stage 3 or 4), which means their lymphoma is widespread. Both ALK-positive and ALK-negative ALCL commonly cause swollen lymph nodes (glands).

B symptoms’ (fevers, night sweats and weight loss) are common in people with ALCL. These symptoms often appear together.

ALK-positive ALCL often affects extranodal sites (areas outside the lymphatic system), like the skin, lungs, liver, bone and bone marrow (the spongy tissue in the centre of your bones where blood cells are made). This is less common in ALK-negative ALCL.

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Treatment

People with ALCL are usually treated with chemotherapy. However, T-cell lymphomas are rare so it is difficult to determine which treatment gives the best outcome. You might be asked if you would like to take part in a clinical trial to help test new treatments and to find out what the best treatment is for ALCL. You might be offered a targeted drug, which may be through a clinical trial.

Most people with ALCL respond to treatment, particularly younger people. ALCL is also less likely to relapse (come back) than other types of T-cell lymphoma.

The most common chemotherapy regimen (combination of drugs) is CHOP. This is made up of:

  • cyclophosphamide
  • doxorubicin (hydroxydaunorubicin
  • vincristine (Oncovin®)
  • prednisolone (a steroid).

Chemotherapy is given in cycles. The drugs are given on certain days, which are followed by a break in treatment for your body to recover before the next cycle begins.

The number of cycles and type of chemotherapy you have depends on several factors like your age, the stage of your lymphoma and whether it is ALK-positive or ALK-negative.

Some people with early-stage ALK-positive ALCL are given fewer cycles of CHOP than usual: 3 or 4 cycles compared with 6–8 cycles for advanced-stage ALK-positive ALCL. Some people with early-stage ALK-positive ALCL also have radiotherapy to the affected area.

More intensive chemotherapy regimens are used for children and young adults with ALCL. Treatment is generally very successful in this group. Research is now focusing on whether less intensive treatment can be used for some people to reduce side effects

Most people with ALK-negative ALCL are treated with CHOP. However, your doctor might consider a more intensive chemotherapy regimen, perhaps adding the drug etoposide (CHOEP regimen) or using a regimen that includes gemcitabine. 

If you are well enough and your lymphoma has been reduced by chemotherapy, your doctor is likely to recommend you have more chemotherapy together with a stem cell transplant. This is to give you a better chance of staying in remission (no evidence of lymphoma) for a long time. ICE chemotherapy (ifosfamide, carboplatin and etoposide) is often used before a stem cell transplant, but your doctor might suggest a different regimen.

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Relapsed and refractory ALCL

If your lymphoma is refractory (doesn’t respond) to treatment or if it relapses (comes back), there are several possible treatment options:

  • a targeted drug called brentuximab vedotin. This is an antibody-drug conjugate, where a strong chemotherapy drug is joined to an antibody that targets a protein called ‘CD30’ found in the lymphoma cells in ALCL. It delivers the chemotherapy directly to the lymphoma cells.
  • a different chemotherapy regimen, eg GEM-P (gemcitabine, cisplatin and methylprednisolone).
  • an allogeneic (donor) stem cell transplant if your lymphoma is reduced by chemotherapy and you are well enough.
  • a different targeted drug, usually through a clinical trial.

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Research and targeted treatments

There are several targeted drugs that can be effective in treating ALCL, either on their own or together with chemotherapy. These are usually only available if you are taking part in a clinical trial because they are still being investigated. You can find out more about clinical trials and search for a trial that might be suitable for you at Lymphoma TrialsLink.

The newer drug brentuximab vedotin is being tested to see if it can help people with ALCL when it is used as a first treatment.

Other drugs being tested in ALCL but not yet approved in the UK, include:

  • ALK inhibitors, like crizotinib, for ALK-positive ALCL.
  • Cell signal blockers that disrupt signals that keep cancer cells alive or allow them to grow. They act on different pathways in cells
    • HDAC inhibitors like belinostat (Beleodaq®) or romidepsin (Istodax®), both of which are already approved in the US for use in people with relapsed or refractory T-cell lymphoma.
    • PI3-K inhibitors like duvelisib.
    • PD1 or PD-L1 inhibitors like nivolumab and avelumab.
  • Immunomodulatory drugs like lenalidomide (Revlimid®) and thalidomide. These affect the activity of the immune system in several different ways. They can help the immune system to attack the lymphoma cells and prevent the lymphoma from growing.
  • Proteasome inhibitors, like bortezomib (Bortezomib Accord or Velcade®) and carfilzomib. These disrupt the balance of proteins in cells, causing them to die.

New drugs are being developed and tested all the time. This is not intended to be a comprehensive list of drugs in development.

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Follow-up

When your treatment is finished, you have regular follow-up appointments at the hospital. People who stay in remission after treatment for ALCL are usually followed up for around 3 years after the end of their treatment.

You should contact your medical team or GP if you develop any symptoms of lymphoma or have other concerns between your appointments. Your specialist might bring your appointment forward if they think they need to see you sooner.

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Further reading

Related content

Lymphoma TrialsLink

Find out more about clinical trials and search for a trial that might be suitable for you.