What to know about T-cell lymphoma

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T-cell lymphoma is an umbrella term for cancers affecting T-cells. T-cell lymphoma can develop from precursor or immature cells (blasts) or mature cells.

T-lymphoblastic lymphoma

This type of lymphoma affects immature forms of T-cells and constitutes 1% of all lymphomas. It is a fast-growing cancer that tends to affect younger individuals and males.

Peripheral T-cell lymphoma (PTCL)

More modern classifications refer to PTCL as mature T-cell lymphoma (MTCL), as it affects mature forms of T cells.

The 2016 World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues lists 29 types of PTCL. These include:

• Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS): This is the most common subtype of PTCL, and it includes all PTCLs that do not fit the other groups. It usually affects older adults and tends to spread and grow quickly.
• Anaplastic large cell lymphoma (ALCL): This typically fast-growing type of lymphoma represents 2% of all lymphomas. It is more common in young people, including children. ALCL tends to respond well to treatment and has three different forms.
• Angioimmunoblastic T-cell lymphoma (AITL): This type makes up about 4% of all lymphomas and is more common in older adults. People with AITL may have spleen and liver enlargement, and they may lose weight. They also tend to develop fevers, infections, and skin rashes.
• Extranodal natural killer (NK)/T-cell, nasal type: This rare type mostly affects the nose and throat, but it may also affect other parts of the body, such as the digestive tract and the skin.
• Enteropathy-associated intestinal T-cell lymphoma (EATL): This type typically appears in the small intestine. It is common among people who develop celiac disease as adults and is more likely to affect males than females.
• Cutaneous T-cell lymphoma (CTCL): This type of lymphoma starts in the skin and makes up about 5% of all lymphomas. Common types include Sézary syndrome and mycosis fungoides.
• Adult T-cell leukemia/lymphoma: This disease occurs as a result of the human T-lymphotropic virus type 1 (HTLV-1), which can transmit in bodily fluids, such as blood and semen. It is common in Japan and the Caribbean islands but rare in the U.S. Learn more about adult T-cell leukemia here.

T-cell lymphoma has a vast range of symptoms, which vary among the types.

However, the characteristic symptoms typically present late in the disease when it is already in the aggressive stages. The initial symptoms often overlap with those of other, benign conditions, making diagnosis challenging.

B symptoms

People with lymphoma may show general, nonspecific symptoms called “B symptoms.” These include:

• fevers that come and go without infection
• night sweats
• rapid weight loss, which refers to an unintentional loss of 5% or more of body weight within 6–12 months

Swollen lymph nodes

A person may see or feel lumps on certain parts of the body where lymph nodes are present.

These areas include:

• the groin
• the sides of the neck
• the underarms
• above the collarbone

These are usually not painful compared with swollen lymph nodes that occur because of an infection, which are known as reactive nodes.

Skin rashes

People with CTCL may initially notice itchy, dry patches that can appear red or purple, depending on a person’s skin tone. Some of these patches may thicken and cause the skin to break. As the disease progresses, small bumps and nodules may appear.

Learn more about lymphoma rashes here.

Neurological symptoms

Lymphoma that affects the brain can cause headaches, difficulty paying attention and processing thoughts, mood and personality changes, and seizures.

Lymphoma can also occur in areas near the brain and spinal cord, causing symptoms such as slurred speech, double vision, and facial numbness. Weakness or numbness of other body parts can also happen.

Abdominal symptoms

Lymphoma in the abdomen can make the liver and spleen swell, causing abdominal pain. An enlarged spleen may make a person feel full easily or cause them to lose their appetite. Lymphoma in the stomach may result in vomiting and nausea.

Chest symptoms

Lymph nodes in the chest may grow and cause chest pain or pressure. They can also press on the windpipe and cause coughing or breathing problems.

Sometimes, the lymphoma can press on the superior vena cava (SVC), causing blood to pool back in the veins. This may lead to SVC syndrome, a life threatening condition.

Other symptoms

Extranodal NK/T-cell lymphoma may cause nasal obstruction, nosebleeds, or nasal bone destruction. People with adult T-cell lymphoma may have hypercalcemia and areas of bone destruction called lytic bone lesions.

In most cases, the cause of T-cell lymphoma is unknown. However, experts associate CTCL with the dysregulation of certain genes and signaling pathways. Some reports also link it with chronic skin inflammation.

Certain types of T-cell lymphoma may be due, at least in part, to viral exposure. For instance, adult T-cell leukemia/lymphoma is linked with the HTLV-1 virus. In 66–99% of AITL cases, people have previously contracted the Epstein-Barr virus (EPV).

A 2018 study suggests that adults aged 50 years and above with celiac disease have a higher risk of developing EATL.

Additionally, a family history of myeloma and T-cell activating autoimmune disease increases a person’s chance of having T-cell lymphoma.

After asking a person about their medical history and symptoms and examining them for physical signs, a doctor will likely recommend further lab testing.

They will generally request a biopsy of the lymph node for lab testing. If the symptoms indicate that lymphoma is present in other parts of the body, they may extract fluid samples to check for lymphoma cells. They may use:

• cerebrospinal fluid
• pleural fluid
• peritoneal fluid

Pathologists will carry out several tests on the samples to diagnose lymphoma and determine the type. Tests include:

• Immunophenotyping: This test looks at specific markers or antigens on the surface of cancer cells to identify the type of lymphoma.
• Chromosome tests: Tests such as molecular cytogenetics, fluorescent in situ hybridization (FISH), and polymerase chain reaction (PCR) tests check for genetic and chromosomal abnormalities that can help identify the type of T-cell lymphoma and aid in treatment planning.

Doctors may also request imaging tests, such as a chest X-ray, CT scan, MRI scan, PET scan, or ultrasound. These can help them find possible causes of the symptoms, determine the extent of the lymphoma, monitor treatment progress, and, at a later stage, check for the recurrence of the disease.

Treatment options for this type of lymphoma include:

CTCL treatment

Treatment for CTCL consists of topical therapy and systemic therapy. Topical therapy includes:

• topical retinoids
• topical chemotherapy
• topical corticosteroids
• topical immunotherapy

Other skin-directed treatment options are radiation therapy, which includes electron beam radiation and total skin electron beam (TSEB) therapy, and phototherapy, which may use UVA, UVA with psoralen (PUVA), or UVB.

A 2021 review states that novel treatments for CTCL, including targeted therapies and histone deacetylase and mTOR inhibitors, have potential benefits for people with this disease.

PTCL treatment

As PTCL is a rare disease, treatments are based on the findings of clinical trials, and experts are still looking for novel therapies for the condition. However, since it is a fast-growing disease, people with PTCL usually receive intensive chemotherapy. The standard first-line chemotherapy treatment for PTCL is CHOP, which stands for:

• cyclophosphamide
• hydroxydaunorubicin
• oncovin
• prednisone

Other regimens, which include the drug etoposide, are CHEOP and EPOCH. These treatments are more suitable for young individuals.

However, a 2019 review suggests that adding etoposide to a CHOP regimen does not significantly change the therapeutic effects.

Another chemo combination option for some of these lymphomas is:

• cyclophosphamide
• doxorubicin
• prednisone
• brentuximab vedotin

Doctors give less intensive drugs, such as gemcitabine and bendamustine, to individuals who cannot tolerate intensive chemo.

They may sometimes recommend autologous stem cell treatment (ASCT) to individuals who have responded well to initial treatment. ASCT may lead to complete remission in certain types of T-cell lymphoma.

Lymphomas in localized areas may be treatable with radiation. Doctors may recommend surgery to remove certain parts of the intestine.

T-cell lymphoma generally has less favorable outcomes than its B-cell counterparts. However, the presence of specific markers affects outlook. For example, having Ki-67, EBV, or CD 26 is associated with a less positive outlook, while AKL and TCR BF1 indicate a better outlook.

A person’s outlook also depends on factors such as their age and the type and stage of T-cell lymphoma. Doctors will also take into account extranodal involvement and lactate dehydrogenase (LDH) levels.

CTCL are lifelong conditions that tend to recur when people stop getting treatments. Over time, these diseases tend to stop responding to treatment despite the various options available.

People in the initial stages of CTCL have a similar life expectancy as healthy individuals, and many people with CTCL die from unrelated diseases such as infections. People with SS are less likely to have a good outlook, as the disease has a median survival rate of 2–4 years.

T-cell lymphoma is an umbrella term for a rare group of blood cancers affecting the immune cells commonly present in lymphoid tissues. The type of T-cell lymphoma will determine the symptoms, management, and outlook.

T-cell lymphoma can be challenging to diagnose. However, anyone who experiences fever, weakness, and sudden weight loss alongside swollen lymph nodes should speak with a doctor about undergoing medical tests.