Anaplastic large cell lymphoma (ALCL) is a rare type of T-cell lymphoma. It accounts for about 1–2% of all cases of non-Hodgkin lymphoma (NHL) in adults. ALCL usually starts in T cells but, in some cases, it is hard to tell what type of cell the cancer started in. This is called null-cell type.
ALCL can occur in any age group, but is more common in children and young adults. It makes up about 14% of childhood NHL cases. It affects more males than females.
There are 2 types of ALCL. ALCL can appear only in the skin (primary cutaneous ALCL) or in organs throughout the body (primary systemic ALCL). A high number of ALCLs are associated with translocations involving the ALK (anaplastic lymphoma receptor tyrosine kinase) gene. ALK-positive tumours (sometimes referred to as ALKomas) have a better outcome than ALK-negative ALCLs. People whose lymphomas express ALK are usually younger. ALK-negative ALCL is more common in older adults and often has an aggressive course.
When ALCL starts in and is confined to the skin, it is called primary cutaneous anaplastic large cell lymphoma. People with primary cutaneous ALCL usually have a single lump or tumour in the skin. It may also spread to lymph nodes in the area. It is associated with a rare skin condition called lymphomatoid papulosis.
Primary cutaneous ALCL is less aggressive than primary systemic ALCL. In general, people with primary cutaneous ALCL have a fairly good prognosis.
Occasionally people with primary cutaneous ALCL have a spontaneous remissionspontaneous remissionA decrease in or the disappearance of signs and symptoms of a disease (such as cancer)..
Primary systemic ALCL usually involves the lymph nodes. It can also occur in organs or tissues other than the lymph nodes (extranodal sites), including:
Primary systemic ALCL is usually a fast-growing (aggressive) lymphoma. Most people have advanced stage (stage III or IV) disease when they are diagnosed.
The lymphoma cells involved in ALCL have a certain marker on their surface called the CD30 antigen. They also express the anaplastic lymphoma kinase (ALK). People with ALK-positive disease respond to chemotherapy and have a more favourable outcome than people with ALK-negative lymphoma. Many people with ALK-negative ALCL will relapse and may need more aggressive chemotherapy. Primary cutaneous ALCL is usually ALK negative, whereas primary systemic ALCL can be ALK positive or negative.
Many people with ALCL can be successfully treated, even with advanced stage disease. Treatment varies slightly depending on the type and extent of ALCL.
ALCL usually responds well to chemotherapy, so it often has a good prognosis. It is treated with the same combinations of chemotherapy drugs used to treat other aggressive lymphomas, including CHOP:
External beam radiation therapy may be used when the lymphoma cells are only in one area of the lymph nodes (stage I primary systemic ALCL) or for localized skin lesions in primary cutaneous ALCL.
Radiation therapy is most often given after chemotherapy.
A stem cell transplant (SCT) may be offered to some people with ALCL who relapse after initial treatment.
The most common treatment options for primary cutaneous ALCL include:
Systemic chemotherapy is only used for people with extensive disease, where many areas of the skin are affected and the person cannot be treated with localized treatments.
Treatment of primary systemic ALCL usually involves chemotherapy. CHOP chemotherapy is used for primary systemic ALCL. Other treatments include radiation therapy and sometimes a stem cell transplant.
Brentuximab vedotin (Adcetris) is a monoclonal antibody that may also be given for systemic ALCL. Health Canada recently approved this drug for people who have not responded to at least one combination chemotherapy regimen.
For more detailed information on specific drugs, go to sources of drug information.