Peripheral T-cell lymphomas

Peripheral T-cell lymphomas (PTCLs) is a group of rare types of non-Hodgkin lymphoma (NHL). They are rare because they start in T cells, while most lymphomas start in B cells. T-cell lymphomas are usually spread throughout the body (widespread) when they are diagnosed.

The following are some of the many different subtypes of PTCLs.

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), is the most common type of PTCL. It is a group of T-cell lymphomas that don't fit into any of the other categories of PTCL.

PTCL, NOS, is most often found in the lymph nodes. It can also be found in extranodal sites, such as the bone marrow, gastrointestinal (GI) tract, liver and skin.

Anaplastic large-cell lymphoma (ALCL) is an aggressive (fast-growing) type of peripheral T-cell lymphoma. ALCL cells have higher than normal amounts of a protein on their surface called CD30, which is involved in cell growth and survival. The higher level of CD30 can help diagnose the disease and treatment for ALCL targets the CD30 protein.

ALCL can occur all over the body (systemic ALCL), in the skin (primary cutaneous ALCL) or in the tissues around breast implants (breast implant–associated ALCL).

Systemic ALCL is grouped based on if there is a certain gene change (mutation) in the lymphoma cells. This gene mutation causes the cells to make a lot of a protein called anaplastic lymphoma kinase (ALK).

• ALK-positive ALCL means that the ALK gene mutation is present. This type is more common in children and younger adults. It responds well to treatment with chemotherapy and targeted therapies.
• ALK-negative ALCL means that the ALK gene mutation is not present. This type is more common in older adults. While this subtype responds to treatment, it is more likely to come back (relapse) after treatment.

Primary cutaneous ALCL starts as itchy spots on the skin that may break open and bleed (ulcerate). These spots can develop slowly into tumours, and may be present a long time before the disease is diagnosed. It doesn't spread from the skin to other parts of the body very often. Primary cutaneous ALCL is almost always ALK-negative.

Breast implant–associated ALCL (BIA-ALCL) is a very rare type of NHL that can develop in people with breast implants (silicone and saline). It starts in the scar tissue that forms around the implant and can cause breast pain, lumps or swelling. Research suggests that the risk for BIA-ALCL is related to textured implants rather than those with smooth surfaces. In 2019, Health Canada suspended the medical license for the one brand of textured breast implants that was available in Canada.

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive (fast-growing) type of peripheral T-cell lymphoma. It may occur in the spleen, bone marrow and liver, as well as in the lymph nodes. It can cause problems with the immune system and can seem like certain immune disorders. Symptoms include rash, fever, weight loss, drenching night sweats and swelling (edema).

Enteropathy-associated T-cell lymphoma is an aggressive (fast-growing) type of peripheral T-cell lymphoma. It occurs most often in people between 60 and 70 years of age. People with celiac disease have an increased risk of developing enteropathy-associated T-cell lymphoma.

Symptoms of enteropathy-associated T-cell lymphoma include stomach pain, weight gain, bleeding in the gastrointestinal (GI) tract and holes in the wall of the small intestine or the colon (called bowel perforations).

Extranodal nasal natural killer (NK)/T-cell lymphoma is an aggressive (fast-growing) type of peripheral T-cell lymphoma. It usually starts in the natural killer cells (NKs) of the immune system. An NK cell is a type of lymphocyte (white blood cell) that attacks abnormal or foreign cells, including cancer cells or cells infected with a virus.

Extranodal nasal NK/T-cell lymphoma occurs in the nose, the nasal passages or both. It can also affect the paranasal sinuses around the nose, the throat and other areas of the body, including the skin, testicles, and gastrointestinal (GI) tract.

Symptoms of extranodal nasal NK/T-cell lymphoma include a blockage in the nose, discharge from the nose or nosebleeds and swelling of the face.

People with extranodal nasal NK/T-cell lymphoma, as well as other types of T-cell lymphoma, often develop a serious condition called hemophagocytic syndrome. It causes certain parts of the immune system to become overactive, which can cause:

• fever
• a larger than normal liver
• low blood cell counts

Hepatosplenic lymphoma is a very rare and aggressive (fast-growing) type of peripheral T-cell lymphoma. It starts in the liver or spleen and can spread to the bone marrow. Unlike other types of NHL, hepatosplenic lymphoma rarely spreads to the lymph nodes. Some research suggests a history of childhood inflammatory bowel disease may increase the risk for hepatosplenic lymphoma.

Hepatosplenic lymphoma can cause low blood cell counts and sores on the skin.

Adult T-cell leukemia lymphoma (ATLL) is a rare type of peripheral T-cell lymphoma. ATLL cells are found in the blood and lymph nodes or other parts of the lymphatic system. Symptoms of ATLL include fatigue, skin rash and enlarged lymph nodes.

The main risk factor for developing ATLL is infection with the human T-cell leukemia/lymphoma virus type 1 (HTLV-1). Most people infected with HTLV-1 will not develop ATLL and more research is needed to know why this happens. Find out more about HTLV-1.

Your healthcare team will create a treatment plan just for you. The plan is based on your health and specific information about the cancer. What you want is also important when planning treatment. When deciding which treatments to offer for peripheral T-cell lymphoma, your healthcare team will consider:

• the subtype of PTCL
• the stage
• your age
• your overall health
• any medical problems you may have
• your lifestyle and what you prefer or want

Chemotherapy uses drugs to destroy cancer cells. The drug combinations commonly used to treat most subtypes of PTCL are:

• CHOP – cyclophosphamide (Procytox), doxorubicin, vincristine and prednisone
• CHOEP – cyclophosphamide, doxorubicin, vincristine, etoposide (Vepesid) and prednisone
• CHP-BV – cyclophosphamide, doxorubicin, prednisone and the targeted therapy drug brentuximab vedotin (Adcetris)
• GOLD – gemcitabine, oxaliplatin, L-asparaginase and dexamethasone
• SMILE – dexamethasone, methotrexate, ifosfamide (Ifex), L-asparaginase and etoposide

If PTCL comes back after treatment or doesn't respond to treatment, the following chemotherapy drugs or combinations may be offered:

• GDP – gemcitabine, dexamethasone and cisplatin
• ICE – ifosfamide, carboplatin and etoposide
• pralatrexate (Folotyn)

Other chemotherapy drugs or chemotherapy drug combinations may be given depending on the type of PTCL.

Find out more about chemotherapy for NHL.

Targeted therapy uses drugs to target specific molecules (such as proteins) on cancer cells or inside them. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells. Targeted therapy may also be called molecular targeted therapy.

Brentuximab vedotin (Adcetris) targets the CD30 protein on the surface of some lymphoma cells. It may be used along with chemotherapy to treat PTCL that expresses CD30, including ALCL, AITL and PTCL, NOS.

Find out more about targeted therapy for NHL.

A stem cell transplant replaces stem cells. It may be offered when PTCL goes into remission after chemotherapy or if PTCL comes back after treatment.

Find out more about stem cell transplant for NHL.

Talk to your doctor about clinical trials open to people with NHL in Canada. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.