Non-Hodgkin lymphoma

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Anaplastic large cell lymphoma

Anaplastic large cell lymphoma (ALCL) is a rare type of T-cell lymphoma. ALCL can occur at any age, but it is more common in children and young adults. It affects more men than women.

Rarely, ALCL can develop in the scar tissue around a breast implant. This is called breast implant associated ALCL (BIA-ALCL). Recent research suggests that the risk is higher in women who have textured implants rather than those with smooth surfaces. Health agencies around the world are still collecting more information about how many women are diagnosed with BIA-ALCL.

ALCL is grouped based on if there is a certain gene change in the lymphoma cells. The change in the gene causes the cells to make a lot of a protein called anaplastic lymphoma kinase, or ALK1. The lymphoma cells with this gene change have a marker on the surface called the CD30 antigen, which is also known as Ki-1 antigen. If the ALK1 gene change is present, it is called ALK-positive ALCL. If this gene change is not present, it is called ALK-negative ALCL.

The average age of people diagnosed with ALK-positive ALCL is 34. The average age of those diagnosed with ALK-negative ALCL is 54. ALK-positive ALCL tends to be fast growing (aggressive). It usually responds well to chemotherapy and has a better outcome than ALK-negative ALCL.

ALCL is divided into 2 types based on where the lymphoma cells are found in the body.

Primary cutaneous ALCL

Primary cutaneous ALCL starts in the skin. The lymphoma cells are only found in the skin, and there is usually only one lump or a few lumps or tumours in the skin. It is linked with a rare non-cancerous skin condition called lymphomatoid papulosis, which can develop into some types of NHL, including ALCL. Primary cutaneous ALCL may spread to nearby lymph nodes, but it is less aggressive than primary systemic ALCL.

Primary cutaneous ALCL is treated in the same way as cutaneous T-cell lymphomas.

Primary systemic ALCL

Primary systemic ALCL usually affects the lymph nodes. It can also develop in organs or tissues other than the lymph nodes (called extranodal sites). These sites include the lungs, liver, bone marrow, bones, skin and soft tissues.

Symptoms

Most people have advanced disease when primary systemic ALCL is diagnosed. They usually have B symptoms, which are unexplained fever, drenching night sweats and unexplained weight loss.

Treatments

Many people with primary systemic ALCL can be successfully treated even when the disease is at an advanced stage. Treatment varies slightly depending on the extent of ALCL.

Chemotherapy is used to treat primary systemic ALCL. The most common chemotherapy combination given is CHOP, which is cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone.

Radiation therapy is sometimes used for ALCL. External beam radiation therapy may be used when the lymphoma cells are only in one group of lymph nodes.

Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop cancer cells from growing and spreading while limiting harm to normal cells. Brentuximab vedotin (Adcetris) is the targeted therapy drug that may be given for systemic ALCL that comes back (recurs) or doesn’t respond to CHOP or another combination of chemotherapy drugs.

Stem cell transplant may be offered if primary systemic ALCL comes back after other treatments.

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