Anaplastic Large Cell Lymphoma (ALCL)

What is anaplastic large cell lymphoma (ALCL)?

Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma that affects the T lymphocytes of the immune system. T lymphocytes travel through the body's lymphatic vessels. Because of this, ALCL may form in almost any location in the body. Common sites include:

• Lymph nodes
• Bones
• Skin
• Lungs
• Muscle
• Abdomen (belly)
• Rarely, the bone marrow and central nervous system

ALCL makes up about 10% of all childhood non-Hodgkin lymphoma cases.

• Weak immune system
  
• Infection with Epstein-Barr virus or human immunodeficiency virus (HIV)
• A genetic disorder such as ataxia-telangiectasia
• More common in males than females
• More common in White than Black children

Diagnosis of anaplastic large cell lymphoma

A biopsy is a sample of tissue taken from a tumor. It helps the care team diagnose your child's condition. 

If the pathologist finds lymphoma cells in the biopsy sample, the lab will perform more lab tests on the sample. These may include flow cytometry, immunophenotyping, and cytogenetic analysis, if these tests are available. Your care provider may order genetic tests to find any genetic changes in the cancer cells.

Types of anaplastic large cell lymphoma

There are 2 types of ALCL:

• Cutaneous ALCL grows slowly and appears on the skin as a red, raised rash or sores.
• Systemic ALCL can occur throughout the body. It has 2 subtypes, based on where there is a change in a gene called ALK:
  • ALK-positive is found mostly in children and young adults.
  • ALK-negative usually occurs in older patients.

Stages of anaplastic large cell lymphoma

Treatment of anaplastic large cell lymphoma

Treatment depends on the following:

• Type of lymphoma and any changes to the genes
• Stage of lymphoma
• How well it may respond to treatment
• Available treatments
• Patient age and health

Treatment may include:

• Chemotherapy 
• Immunotherapy

As with any treatment, there can be side effects. One problem is tumor lysis syndrome. This happens when cancer cells quickly die and break apart in response to treatment. The care team will watch your child closely for possible side effects. Speak with your health care provider if you have questions.

Newly diagnosed disease

Examples of commonly used medicines to treat ALCL include: 

• Cyclophosphamide
• Ifosfamide
• Doxorubicin
• Prednisone
• Dexamethasone
• Vincristine
• Vinblastine
• Methotrexate
• Cytarabine
• Etoposide
• Brentuximab vedotin

Chemotherapy usually takes 3–12 months to complete.

Relapsed disease

For relapsed ALCL, treatment may include:

• Chemotherapy 
• Targeted therapy (such as crizotinib)
• Immunotherapy (such as brentuximab, antibody therapy, or an anticancer drug combined with an antibody against a common protein found on ALCL cells called CD30
• Stem cell (bone marrow) transplant 

Prognosis for anaplastic large cell lymphoma

Your health care provider is the best source of information about your child's case. Each case is different, and the prognosis depends on a number of factors, including:

• Stage of lymphoma
• Where the disease is located

The 5-year event-free survival rate for ALCL in the United States is about 70%. Children with ALK-positive ALCL usually do well with chemotherapy. Those with ALK-negative ALCL have a greater risk of relapse.

Support for anaplastic large cell lymphoma

Coping with a cancer diagnosis and treatment can be stressful for you and your family. You may want to talk to a social worker, a psychologist, or another mental health specialist.

Learn more about how to talk to your child about cancer.

After treatment, your child's care team may use imaging tests and exams to watch for recurrence. Childhood cancer survivors should get long-term follow-up care. Some treatments can cause late effects. These are health problems that happen months or years after the end of their treatment. 

After completing treatment, it is important that your child:

• Has regular checkups and screenings by a primary care provider
• Maintains healthy habits to protect their health, including physical activity and healthy eating
• Has a survivorship care plan to share with their health providers, which includes:
  • Guidance on health screenings
  • Disease risk factors
  • How to improve health

Questions to ask your care team

• What does the biopsy show?
• What stage of cancer does my child have?
• What are my child's treatment options?
• What are the possible side effects of each treatment?
• How can my child manage their side effects?
• Will my child need to be in the hospital for treatment?
• Where is the treatment available? Is it close to home or will we have to travel?
• What resources are available to help us cope with this illness?
• What monitoring does my child need after treatment?
• How can I help my child stay healthy?
• What are the possible late effects of treatment?

Find out more information