T Cell Lymphoma

August 15, 2024

This page was reviewed under our medical and editorial policy by Alex Herrera, M.D., associate professor, Division of Lymphoma, Department of Hematology & Hematopoietic Cell Transplantation, City of Hope^® Cancer Center Duarte

T cell lymphomas are a rare type of non-Hodgkin lymphoma that may develop inside lymphatic tissues, including the spleen and lymph nodes, or outside these tissues in areas such as the digestive tract or liver. It is an uncommon type, accounting for less than 15% of all non-Hodgkin lymphomas in the United States.

What Is T Cell Lymphoma?

The lymphoid system is the foundation of the body’s immune response. T cells are one of several types of white blood cells that help fight infection. T cell lymphoma is a disorder in which these particular immune cells become dysfunctional, and it includes several subtypes. Some of the subtypes of T cell lymphoma may be very slow to spread, while other types may be aggressive.

T Cell Lymphoma Symptoms

T cell lymphoma symptoms may vary, depending on where the condition starts. The first symptom may be the enlargement of one or more lymph nodes, which may cause a bump under the skin. Common areas are on the side of the neck, under the arm (armpit), under the collarbone or in the groin area. Some symptoms may resemble other medical conditions, so it is important to speak with a health care provider if these symptoms appear.

Other symptoms may include those listed below.

• Night sweats: These intense sweating episodes and/or hot flashes occur at night.
• Fever: This elevated body temperature is not related to a short-term illness/virus. This may be accompanied by chills, shaking or body aches.
• Unexplained weight loss: Losing weight without changing diet or exercise habits may be a symptom. This is commonly defined as losing between 5% and 10% or more of body weight within six to 12 months.
• Swollen lymph nodes: Enlarged lymph nodes may be present in the neck (below the ear), under the collarbone or in the armpits or groin.

What Causes T Cell Lymphoma?

T cell lymphoma does not have a single cause. Typically, it occurs when the genes that regulate T cell formation mutate, causing cells to develop abnormally. These abnormal cells do not work the way they are intended. However, the specific cause of one patient’s illness may not be pinpointed.

T Cell Lymphoma Risk Factors

Certain risk factors may increase the chances of developing a disease such as cancer, but having a risk factor does not mean a person will get the disease. Some risk factors (such as tobacco use) may be within a person’s control, while others, such as age or family history, are not.

The risk factors for T cell lymphoma are listed below. Some are known, such as older age and male gender, while many others are still under investigation.

Age: Most lymphomas occur after age 60. Certain lymphomas, however, are more prevalent in youth.

Sex: Men are more likely to develop T cell lymphoma than women.

Race: White people are more likely to develop T cell lymphoma than people who are Asian or Black.

Having an autoimmune disease: People with a weakened immune system are at increased risk for several types of cancers, including T cell lymphoma.

Exposure to some chemicals and drugs: Studies have linked non-Hodgkin lymphoma to chemicals such as benzene and some herbicides and insecticides. Researchers continue to clarify this potential link.

Certain infections: Some infections, including HIV/AIDS, Epstein-Barr virus and the herpes virus HHV-8, may increase the risk of non-Hodgkin lymphoma.

Body weight: Although more study is needed to confirm this, some research has suggested that being overweight or obese may increase the risk for non-Hodgkin lymphoma.

Breast implants: It is rare, but the scar tissue surrounding breast implants (capsule) may develop lymphomas. Most of these are considered breast implant-associated anaplastic large cell lymphoma (BIA-ALCL), though there are other, less common types.

T Cell Lymphoma Diagnosis

T cell lymphoma may be diagnosed in a variety of ways, and more than one test is often required to make an official diagnosis. The process usually starts with a thorough health history and a physical examination. The exam will be used to detect the presence of enlarged lymph nodes, skin lesions or any other abnormal findings.

Several tests may also aid in diagnosis. They include those listed below.

Biopsy

A biopsy is the only way to confirm that cancer is present, and there are several ways it may be performed. During a biopsy, a small sample of tissue in the suspicious area is obtained for testing. Then, a pathologist examines the sample and determines which type of cancer cells are present. Common biopsy procedures include those listed below.

• Needle biopsy: The doctor uses a hollow needle attached to a syringe to remove a small sample of tissue from a suspicious area for analysis.
• Incisional or excisional biopsy: This is the most common type for evaluating suspected lymphomas. In the procedure, all or part of a lymph node is removed for analysis.
• Bone marrow aspiration and biopsy: These procedures are often performed together. With the patient under local or general anesthesia, samples of liquid and solid marrow are removed, usually from the hip, using a needle and syringe.
• Lumbar puncture (spinal tap): This test may be ordered if cancer is suspected to have reached the brain. A small, hollow needle is used to extract cerebrospinal fluid from between bones of the lower spine.

The tissue samples may be tested in several ways to identify the type of lymphoma. Flow cytometry and immunohistochemistry help to pinpoint specific proteins on cells. In addition, cytogenetic analysis, fluorescent in situ hybridization (FISH) and polymerase chain reaction (PCR) tests help to identify chromosome changes that correlate with different types of lymphomas.

Imaging Studies

Imaging studies may be done to determine the exact location of the body that is affected by lymphoma and to determine the stage. The most common ones include those listed below.

Chest X-ray: This test provides an inside view of the chest to look for enlarged lymph nodes.

Computed tomography (CT) scan: These show far more detail than an X-ray, including details of soft tissues, such as organs. Enlarged organs or lymph nodes may be identified in this way.

CT-guided needle biopsy: A CT scan is used to help guide a biopsy needle through the skin, toward the suspicious area. A tissue sample is removed and sent to a laboratory for examination under a microscope.

Magnetic resonance imaging (MRI) scans: Radio waves and strong magnets are used to show detail in the soft tissues and organs.

Positron emission tomography (PET) scan: The patient is injected with a slightly radioactive glucose (a type of sugar), which is quickly absorbed by cancer cells. A specialized camera then creates a picture of areas where the radioactivity has gathered. The PET scan may help determine whether an enlarged lymph node is cancerous and whether treatments have been successful.

T Cell Lymphoma Subtypes

T cell lymphoma is a less common type of non-Hodgkin lymphoma, making each subtype even more rare.

T Lymphoblastic Lymphoma and Leukemia

Representing about 1% of lymphomas, this type is more common in teens and young adults, and affects males more often than females. It may be considered either a lymphoma or a leukemia, depending on how much bone marrow is affected. This fast-growing cancer often starts in a small organ behind the breastbone called the thymus. Large tumors between the lungs may cause breathing issues and swelling in the face and arms. Chemotherapy may successfully treat it if it is found before it has reached the bone marrow.

Peripheral T Cell Lymphoma Not Otherwise Specified (NOS)

These types of lymphomas develop from mature forms of T cells and are predominantly found in adults. 

Cutaneous T cell lymphomas (mycosis fungicides, Sézary syndrome and others): These lymphomas start on the skin and comprise about 5% of all lymphomas.

Adult T cell leukemia/lymphoma: This subtype is caused by a viral infection called HTLV-1. It is rare in the United States, but common in countries where the virus is more prevalent, including the Caribbean, Japan and parts of Africa.

It may affect the bone marrow, lymph nodes, spleen, liver, skin and other organs. It includes the four subtypes listed below.

• Smoldering: It tends to grow slowly, often without symptoms and has a good prognosis.
• Chronic: It is slow-growing, and the prognosis is good.
• Acute: This is the most common subtype. It grows fast and requires quick treatment.
• Lymphoma: This subtype grows faster than chronic and smoldering types, but slower than the acute type.

Angioimmunoblastic T cell lymphoma: About 4% of lymphomas are this type, which is more common in older adults. It usually involves the lymph nodes, bone marrow, spleen or liver, which may become swollen. Common symptoms include weight loss, fever, rashes and recurring infections. Because it may progress quickly, early treatment is often recommended. Though treatment may be successful initially, the lymphoma may return later.

Enteropathy-associated intestinal T cell lymphoma (EATL): This lymphoma mainly affects the lining of the intestinal tract, but it may also be found in the colon. This may occur in some people with celiac disease, an illness caused by sensitivity to gluten. Symptoms include severe abdominal pain, nausea, vomiting and bleeding in the intestine.

Anaplastic large cell lymphoma (ALCL): This type, which accounts for 2% of lymphomas, is more common in the young. Though fast-growing, it may be managed well with treatment.

Extranodal natural killer/T cell lymphoma, nasal type: This rare type usually involves the nose, upper throat and nasal passages, but may include the skin, digestive tract and other organs. It is more common in Asia and South America.

Peripheral T cell lymphoma, not otherwise specified (PTCL-NOS): This subtype is used for lymphomas that do not fit into any other well-defined types, and diagnosis is most common after age 60. These lymphomas usually involve the lymph nodes, but they may also affect the skin, bone marrow, spleen, liver and digestive tract. They are often extensive and grow rapidly.

T Cell Lymphoma Stages

Knowing the stage of lymphoma helps doctors develop more appropriate treatment options.

Today, cancer specialists use the Lugano staging system to determine the extent to which most T cell lymphomas have progressed. The Lugano system uses the diaphragm (the muscle that separates the chest from the abdomen) to divide the body in half by an upper and lower region, and also left and right sides, for staging. Whether the cancer cells are found in lymph tissue only, or in organs outside the lymph system, also help determine the stage.

The stages for T cell lymphoma are listed below.

• Stage 1: This involves one lymph node or lymphatic site, such as the tonsils. In Stage 1E, the cancer is found in an organ outside of the lymph system.
• Stage 2: This involves two or more lymph node regions on the same side of the diaphragm. In Stage 2E, the cancer is found in lymph nodes as well as a nearby organ outside the lymph system.
• Stage 3: This stage involves lymph nodes on both sides of the diaphragm, upper and lower, or affects nodes above the diaphragm and also the spleen.
• Stage 4: One or more organs outside the lymph system is involved, with or without lymph node involvement. This stage includes any spread to cerebrospinal fluid, bone marrow, liver or lung. 

Skin lymphomas are staged with a different system that looks at how much of the skin, lymph nodes, other organs and blood are affected.

T Cell Lymphoma Treatment

The care team will customize the patient’s treatment plan depending on the type of lymphoma and its stage. Some very slow-growing cancers may be watched until treatment is needed.

Chemotherapy: This treatment uses powerful drugs that target fast-growing cells in the body. It works identifying cells that grow and multiply quickly, as cancer cells do, identifying them and interfering with the cell division process. This prevents the cancer cells from reproducing.

Monoclonal antibodies: These antibodies are created in a laboratory to help fight lymphoma cells. Some types recognize targets (similar to the way the natural immune system works) and may help the body fight off cancer. Other types may target specific lymphoma cells, making them more visible to the immune system. Both work by empowering the immune system to fight cancer.

Radiation therapy: This treatment uses high-energy waves to render the lymphoma unable to grow and divide. It is usually given in a series of sessions over time. Like chemotherapy, it may be used alone, or in combination with other treatments.

Stem cell transplant: High-dose chemotherapy or radiation therapy may destroy bone marrow, which is responsible for producing healthy blood cells. The patient may need a stem cell transplant if too many of these blood-forming cells have been damaged. Stem cells may be collected from a donor or taken from the patient before starting chemotherapy. They are then returned to the patient’s bone marrow.

Targeted therapy: Targeted therapy involves the use of drugs designed to “target” parts of cancer cells. The drugs either block signals to multiply or turn on signals telling target areas to destroy themselves.

T Cell Lymphoma Survival Rate

The prognosis (outcome) for T cell lymphoma depends on the type and stage of lymphoma, and the patient’s age when diagnosed.

The overall five-year relative survival rate for this cancer is 63%, according to the National Cancer Institute. This rate varies from more than 90% for mycosis fungoides to less than 15% for enteropathy T cell lymphoma.

A five-year relative survival rate represents the number of patients who were alive five years after their original diagnosis. The rate does not take into account new medications and treatment options that have developed over that period of time. In addition, clinical trials are ongoing and continue to improve the prognosis for T cell lymphomas.