Anaplastic large cell lymphoma: Types, diagnosis, and treatment

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ALCL is a rare type of non-Hodgkin lymphoma (NHL) and a type of T-cell lymphoma. It accounts for around 1% of all NHL cases and roughly 16% of all T-cell lymphoma cases. T cells are a type of cell that forms part of the immune system.

ALCL gets its name from the appearance and behavior of the lymphoma cells and features large cancer cells. “Anaplastic” is the term health experts use to describe cancer cells that look very different from typical cells and divide rapidly.

Another key feature of ALCL cells is that they have a certain protein, called CD30 antigen, on the surface of the cells.

ALCL may occur in the skin, lymph nodes or organs throughout the body, or scar tissue around breast implants.

There are different types of ALCL, depending on where the cancer cells occur in the body. These types are:

Systemic ALCL

Systemic ALCL usually occurs in the lymph nodes but can also begin in other areas of the body, such as the:

• lungs
• liver
• bone marrow
• bones
• skin
• soft tissues

Systemic ALCL divides into either anaplastic lymphoma kinase (ALK)-positive or ALK-negative, depending on whether the ALCL cells produce ALK protein. Systemic ALCL is usually fast-growing.

Cutaneous ALCL

Cutaneous ALCL develops in the skin but may spread to surrounding lymph nodes. This condition is slower growing than systemic ALCL and is usually ALK-negative.

Breast implant-associated ALCL (BIA-ALCL)

BIA-ALCL is a type of ALCL that occurs in people with breast implants. It develops when ALCL forms in scar tissue around breast implants. Textured implants may have a higher risk of BIA-ALCL than smooth implants.

People of any age may develop ALCL, but it more commonly affects children and young adults. It also occurs more frequently in males.

Certain types of ALCL may be more common in certain groups of people:

• Systemic ALK-positive ALCL: This is more common in children and young adults, with an average age at diagnosis of 34 years. It may also affect older adults.
• Systemic ALK-negative ALCL: This is more likely in older adults, with an average age at diagnosis of 58 years. It is slightly more common in males.
• Primary cutaneous ALCL: This is more common in older adults, with an average age of 55 years, as well as males. This type of ALCL rarely occurs in children.

The symptoms of ALCL may depend on the type and stage of cancer but may include:

• swollen lymph nodes, usually around the neck, armpit, or groin, which will feel painless
• fever
• night sweats
• unexplained weight loss
• backache
• loss of appetite
• itching
• skin rash
• fatigue

Symptoms of cutaneous ALCL may include one or more raised, red lesions on the skin that do not go away and may become ulcerous and itch.

Additionally, BIA-ALCL may cause swelling, lumps, or pain in the breast area.

There is currently no known cause for ALCL, but conditions that may have links to the disease include:

• HIV
• mycosis fungoides, a rare type of blood cancer that starts in the skin
• pulmonary and inflammatory pseudotumors

Breast implants may also have associations with BIA-ALCL.

Learn more about breast lymphoma.

To diagnose ALCL, doctors may use the following tests:

• a biopsy of skin or tissue
• blood tests
• a bone marrow biopsy to examine a bone marrow sample
• imaging tests, such as CT, PET, or MRI scans

These tests can also help doctors diagnose the type and stage of ALCL and determine the best treatment approach.

Treatment for ALCL may vary depending on its type and stage. It may include:

• Chemotherapy: To treat systemic ALCL, doctors may use a combination of the chemotherapy drugs cyclophosphamide, doxorubicin, vincristine, and prednisone.
• Radiation therapy: Doctors may use external beam radiation to target ALCL cells in one cluster of lymph nodes.
• Targeted therapy: These drugs target specific proteins in cancer cells to prevent them from dividing and spreading. This method may cause less harm to surrounding healthy cells. Doctors may also recommend brentuximab vedotin, a drug that specifically targets the CD30 antigen in ALCL cells.
• Stem cell transplant: If ALCL returns after treatment, people may require a stem cell transplant.

Cutaneous ALCL may go into remission without treatment but will usually relapse. People may need surgery to remove cutaneous ALCL that is in the skin only.

If the condition spreads to lymph nodes or organs, doctors often treat it the same as systemic ALCL.

Treatment for BIA-ALCL usually involves surgery to remove the breast implants, ALCL cells, and surrounding tissue. In most cases, surgery will cure BIA-ALCL.

If ALCL remains after surgery, people may have additional treatments, such as chemotherapy, radiation therapy, or targeted therapy.

The outlook for ALCL depends on the type:

• ALK-positive ALCL: This is usually fast-growing but often responds well to chemotherapy and may have a better outlook than ALK-negative ALCL. Chemotherapy may lead to long-term remission in ALK-positive ALCL.
• ALK-negative ALCL: This may respond well to treatment at first but may be likely to return within 5 years of treatment.
• Cutaneous ALCL: Around 10% of cutaneous ALCL may spread to lymph nodes or organs.
• Systemic ALCL: In many cases, treatment is successful for this condition, even in the advanced stages of the disease.
• BIA-ALCL: Complete surgical removal of the breast implant and capsule is usually effective in curing BIA-ALCL in most cases.
• Cutaneous ALCL: Around 40% of cutaneous ALCL cases may relapse, but the long-term outlook is more favorable if ALCL only affects the skin. If it only affects one area of the skin, surgery or radiation therapy may result in remission in around 95% of cases.

ALCL is a rare type of non-Hodgkin lymphoma and a form of T-cell lymphoma. Different types of ALCL may affect lymph nodes, organs, skin, or breast tissue.

Treatment options may include chemotherapy, radiation therapy, surgery, and targeted therapy drugs. In some cases, people may require a stem cell transplant.

The outlook for ALCL may depend on the type and stage of the disease, age, and overall health.