Anaplastic large cell lymphoma (ALCL) is a rare form of non-Hodgkin lymphoma. It’s a serious form of cancer that affects cells called T lymphocytes. Some forms of ALCL are considered aggressive and spread quickly. Others grow more slowly. Regardless of what type of ALCL you have, there are treatments like surgery and chemotherapy that can help.
Anaplastic large cell lymphoma (ALCL) is a rare form of non-Hodgkin lymphoma. Like all non-Hodgkin lymphoma, ALCL is a type of cancer. With non-Hodgkin lymphoma, white blood cells called lymphocytes grow out of control. Lymphocytes are part of your immune system. They help protect your body from germs and diseases.
Lymphocytes include two main types of cells: B-cells (B lymphocytes) and T-cells (T lymphocytes). In ALCL, your T lymphocytes grow abnormally. The condition's name — anaplastic large cell lymphoma — describes how the abnormal T lymphocytes look when viewed under a microscope.
The various types of ALCL are classified based on the cancer’s location in your body and the cancer cells’ characteristics. “Systemic” ALCL may affect your lymph nodes and organs, including your skin. Other types of ALCL primarily affect only a particular body part, like your skin or breasts.
Systemic forms of ALCL are classified based on whether there’s a mutation (change) in the anaplastic lymphoma kinase (ALK) gene. Genes are the instructions that tell a cell (like a T lymphocyte) how to perform essential functions. Systemic ALCL that has a mutation on the ALK gene is called "ALK-positive" ALCL. Systemic ALCL that doesn’t have the mutation is called “ALK-negative.”
Other types of ALCL grow more slowly than the systemic types.
ALCL can affect people of all ages, but certain groups are more likely to develop certain types.
Anaplastic large cell lymphoma is rare. Approximately 2% of adult non-Hodgkin lymphoma diagnoses are ALCL.
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Symptoms vary depending on the type of ALCL.
Both ALK-positive and ALK-negative ALCL often cause swollen lymph nodes at the site where the cancer’s growing. The most common areas are your neck, underarm and groin. Other symptoms include:
Most people are diagnosed when the cancer is more advanced. In advanced stages, the cancer may have spread to organs, like your lungs, liver and bone. You may experience symptoms based on which body parts are affected. For example, pressure in your chest and a frequent cough may signify the presence of the ALCL in your chest.
Primary cutaneous ALCL often appears as a single abnormal growth or multiple growths on a single area of your skin. The most common sites are your face, abdomen, buttocks, arms and legs. Rarely (about 20% of the time), growths appear in multiple places. Symptoms include:
BIA-associated ALCL often causes noticeable breast changes. Symptoms include:
Usually, just one breast changes, but you may notice changes in both breasts.
Anaplastic large cell lymphoma forms when lymphocytes multiply out of control and begin to overtake surrounding healthy tissue. Researchers haven’t yet found what causes lymphocytes to become malignant (cancerous). Still, they’ve identified several genetic mutations that frequently occur with ALCL. These mutations may cause healthy cells to transform into cancer cells.
For example, mutations in the ALK gene are the defining feature of ALK-positive ALCL. Other types of gene mutations are common with other forms of ALCL.
Your healthcare provider will perform a physical exam to check for signs of ALCL, such as swelling in your lymph nodes. If they suspect ALCL, they’ll perform various tests and procedures.
Chemotherapy is the standard treatment for systemic ALCL. Surgery is the preferred treatment for primary cutaneous ALCL and BIA-ALCL.
ALK-positive and ALK-negative anaplastic large cell lymphoma usually respond well to systemic chemotherapy. With systemic chemotherapy, drugs that destroy cancer cells are given in a vein and are distributed through your bloodstream to your body, killing cancer cells.
Chemotherapy treatments for systemic ALCL often include a combination of drugs that work together to destroy cancer cells. Drug combinations include:
These treatments usually eliminate all signs of cancer (remission) in the short term. Still, the cancer may return. If your physician believes it’s likely that your cancer will return, they may recommend a stem cell transplant while you’re in remission. During a stem cell transplant, your healthcare provider replaces the cells destroyed during chemotherapy with healthy cells. These cells develop into healthy (noncancerous) blood cells.
If the cancer returns, your provider may recommend various additional chemotherapy treatments designed to treat lymphoma.
Surgery is the most common treatment for primary cutaneous ALCL. You may need radiation therapy if the cancer has spread to your lymph nodes. Radiation directs energy toward the cancer cells, destroying them.
Primary cutaneous ALCL often returns within five years of treatment. You may need additional surgery and radiation therapy if it comes back.
You may need other forms of treatment if the localized tumors can’t be removed with surgery or if your cancer keeps returning. Treatments may include:
Surgery to remove your implant and the surrounding cancer cells usually cures BIA-ALCL. If surgery isn’t an option, you may receive radiation therapy to destroy the cancer cells.
If the cancer returns or spreads beyond your breast, your healthcare provider may recommend chemotherapy treatments used to treat ALK-negative ALCL. Treatments may include CHOP chemotherapy and brentuximab vedotin.
Anaplastic large cell lymphoma is a serious cancer that requires careful monitoring and appropriate treatment, no matter the type. Still, your prognosis and care plan depend on what form of ALCL you have and other factors. These factors include your response to treatment and your score on the international prognostic index (IPI).
Cancer specialists (oncologists) created the IPI to help predict likely outcomes associated with aggressive forms of non-Hodgkin lymphoma, including systemic ALCL. The index assigns a score based on factors like:
Ask your healthcare provider how your IPI score will affect your prognosis if you have systemic ALCL.
The survival rates associated with systemic forms of ALCL vary greatly. ALK-positive ALCL is associated with a longer survival timeline than ALK-negative ALCL. Depending on factors like your cancer type and your IPI score, the five-year survival rate of ALK-positive ALCL ranges from 33% to 90%. Similarly, the five-year survival rate of ALK-negative ALCL ranges from 13% to 74%.
The survival rates for primary cutaneous ALCL and BIA-ALCL are excellent. Although primary cutaneous ALCL usually returns within five years of remission, follow-up treatments can extend your life. The survival rate five years following diagnosis is 80%. With BIA-ALCL, completely removing the cancer surgically often eliminates the cancer for good.
Questions to ask your healthcare provider include:
A note from Cleveland Clinic
Learning of a cancer diagnosis may feel scary. Still, it’s important to remember that ALCL isn’t just one type of cancer with a single prognosis. The kind of ALCL you have will determine whether it spreads quickly or slowly. It will shape your treatment options and your outlook. In some people, ALCL spreads fast. In others, it develops more slowly, allowing plenty of time for you to receive treatments that can send your cancer into remission. Talk to your healthcare provider about likely outcomes based on your unique diagnosis.
Last reviewed by a Cleveland Clinic medical professional on 08/10/2022.
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