All about anaplastic large cell lymphoma (ALCL)
Anaplastic large cell lymphoma (ALCL) is a fast growing cancer that affects T cells. T cells are a type of white blood cell that helps fight infection. In ALCL, T cells grow in an out of control way and do not work properly. These abnormal cells can build up in the lymph nodes or skin (as well as other areas) and cause problems.
There are four types of ALCL:
- systemic ALCL, ALK positive
- systemic ALCL, ALK negative
- primary cutaneous ALCL
- breast implant associated ALCL (BIA-ALCL).
ALK stands for anaplastic lymphoma kinase, which is a gene. If you have ALK positive ALCL, there is a mutation in this gene. This causes cancer cells to grow quickly. Additionally, most ALCL cells have a protein on their surface called CD30. Treatments can target this protein to help kill cancer cells.
Symptoms of ALCL
Symptoms of ALCL vary depending on the subtype.
Symptoms of systemic ALCL
- painless swelling of lymph nodes in the neck, armpit or groin
- feeling tired for no reason
- fever
- night sweats
- unexplained weight loss.
Symptoms of primary cutaneous ALCL
- growths on the skin that get bigger
- changes in skin colour
- raised bumps on the skin that may be itchy
- unexplained skin ulcers and scabs.
Symptoms of breast implant associated ALCL (BIA-ALCL)
- breast pain
- breast swelling
- rash
- unexplained lump near your implant.
Causes of ALCL
In most cases, there is no cause of ALCL.
Some factors that can increase your risk of developing ALCL include:
- being male
- your age for some subtypes
- conditions or treatments that weaken the immune system, such as an HIV infection
- having textured breast implants.
Stages of ALCL
Staging looks at where the lymphoma is in your body, including what organs it might be affecting. Lymphoma cells can travel anywhere in the body.
There are 4 stages of lymphoma. Stage 1 and 2 are early stage disease, and stage 3 and 4 are advanced disease.
Stage 1
Means the lymphoma is in one lymph node area or in one area of a single organ such as the liver.
Stage 2
Affects two or more lymph node areas either above or below the diaphragm or it has spread from one lymph node area to a nearby organ.
Stage 3
Is in lymph node areas on both sides of the diaphragm.
Stage 4
Has spread beyond the lymph nodes to other areas of the body.
The diaphragm is the dome shaped muscle at the bottom of your lungs.
Types of ALCL
- produces a protein called anaplastic lymphoma kinase (ALK) which drives the growth of the cancer cells
- affects pre teens to adults in their 30s
- more common in males.
- mainly affects people over 60
- more common in males.
- affects adults over 40
- more common in males
- more common among Caucasian people.
- affects people in their 50s
- affects people with both silicone and saline textured implants
- typically presents 8 to 10 years after the implantation.
Diagnosis of ALCL
ALCL is diagnosed with tests.
The best test to diagnose ALCL is a biopsy of a lymph node, lump or the affected skin. A sample of tissue is taken from the affected area and looked at under a microscope.
Other tests you may have include:
- medical history and physical exam
- blood tests – full blood count (FBC), kidney and liver function, electrolytes, lactate dehydrogenase, beta 2 microglobulin, hepatitis and human immunodeficiency virus (HIV)
- genetic tests
- chest x-ray
- computed tomography (CT) scan
- positron emission tomography (PET) scan
- ultrasound
- bone marrow biopsy.
These tests help your treatment team work out your stage and prognosis.
Fertility
Some treatments for ALCL can affect fertility. For women some treatments can cause damage to the ovaries. For men it is possible to have low or abnormal sperm production. Your fertility may become normal again in the future, but it is difficult to predict.
It is important to ask your doctor about your risk of infertility as early as possible. There are some options for preserving fertility. Decisions about what options might be right for you usually need to be made before you start treatment.
For more information go to our fertility webpage.
Treatment for ALCL
Treatment for ALCL depends on:
- subtype
- stage
- your age
- your general health.
Chemotherapy (chemo) is the most common treatment for systemic ALCL. Chemo kills cancer cells and stops them from growing. It also damages healthy cells and causes side effects. There is information on chemotherapy side effects and how to manage them on our chemotherapy webpage.
The type of chemo recommended will depend on your subtype of ALCL, treatment goal, age, and medical history. Chemo can be given in combination with targeted therapy.
Treatment plans for ALCL are shortened to letters like CHOP. The letters represent the name of the medications. Your haematologist will give you information on your plan, including when and how often you will have it.
Targeted therapy is a cancer treatment that finds and blocks parts of cancer cells that help them grow and spread.
An antibody–drug conjugate is a type of targeted therapy used to treat systemic ALCL or BIA-ALCL. It combines antibody therapy with chemo to help kill cancer cells.
Brentuximab vedotin is an anti-body drug conjugate used to treat ALCL. It targets a marker called CD30 on some lymphoma cells. It attaches to these cells and delivers chemo into the cell to kill them.
It is usually given through a drip into a vein (IV) in a hospital or clinic. Common side effects includes tiredness, nausea, and tingling or numbness in the hands and feet.
Read more on Immunotherapies and targeted therapies | Leukaemia Foundation.
Your treatment team might suggest surgery to treat your primary cutaneous ALCL or BIA-ALCL. It can be used to remove lesions or lumps from the skin in primary cutaneous ALCL. Additionally, it is the main treatment for BIA-ALCL. This is because the implants are likely causing the cancer. The surgery aims to remove the implants and surrounding tissue.
Following surgery, your treatment team may suggest you have intravenous (IV) treatment. This will depend on how far the disease has spread and if it is affecting other organs.
A bone marrow or blood stem cell transplant is a treatment that restores stem cells after high dose chemotherapy. Your treatment team might suggest a transplant if your ALCL is difficult to treat or has come back after a period of remission.
An autologous stem cell transplant involves collecting your own stem cells and giving them back to you after high dose chemotherapy.
You can read more on our autologous stem cell transplant webpage.
Radiation therapy, also called radiotherapy, is a type of treatment that:
- uses high energy X-rays to kill cancer cells
- is a local therapy because it only destroys cancer cells in the area it treats
- can be used to treat primary cutaneous ALCL.
Find out more about radiation therapy on our webpage.
Topical treatment can be used to treat localised slow growing primary cutaneous ALCL. These include topical steroids, chemotherapy, a type of Vitamin A and laser.
Clinical trials may be available for some types of anaplastic large cell lymphoma. They often compare new or combination therapies with current treatments, including side effects.
Many are randomised, meaning some patients receive the new treatment, and others receive standard care. Trials help improve future treatments and may offer access to costly new therapies not on the Pharmaceutical Benefits Scheme (PBS) in Australia.
If you are considering a clinical trial, your doctor will explain:
- the possible risks and benefits
- how it compares to standard treatment
- what is involved.
You will need to give informed consent before taking part.
Survival rate for ALCL
The survival rate for ALCL ranges from 70% to 90%, five years from diagnosis. However, systemic ALCL, ALK negative disease has a lower survival rate of 30% to 50%. Survival rate is a population-based measure. Whereas your individual prognosis takes into account factors that can impact the survival rate.
Your prognosis is estimated by your haematologist. It is a prediction of the likely course and outcome of your disease. The factors considered when discussing your prognosis include:
- your subtype and stage
- your overall health
- your age.
Your prognosis might change if your ALCL comes back or does not respond to treatment.
Follow up care for ALCL
Follow up care for anaplastic large cell lymphoma includes regular checkups with your treatment team. This is to check for long term side effects. You will also have tests and scans to make sure the lymphoma hasn’t come back (relapsed/recurred). You will likely need more treatment if the lymphoma returns.
Caring for someone with ALCL
We have a range of information and resources that may help when you are caring for someone with anaplastic large cell lymphoma.
Resources for ALCL
Booklets to download:
The non-Hodgkin lymphoma booklet may provide information relevant to anyone diagnosed with anaplastic large cell lymphoma.
The NHL booklet has been translated into the following languages:
References
- Anaplastic Large Cell Lymphoma: Symptoms, Causes & Treatment
- ALK-positive anaplastic large cell lymphoma in adults – PMC
- Anaplastic large cell lymphoma – Survival rates – Mayo Clinic
- Anaplastic Large Cell Lymphoma (ALCL) – Lymphoma Australia
- Primary Cutaneous Anaplastic Large Cell Lymphoma—A Review of Clinical, Morphological, Immunohistochemical, and Molecular Features – PMC
- Primary Cutaneous Anaplastic Large Cell Lymphoma: A Review of Diagnosis and Treatment for the General Oncologist
- 1665-Brentuximab vedotin | eviQ
