T-cell lymphoma: Symptoms, pictures, and treatment – Medical News Today

The immune system produces immune cells that travel through the lymphatic system to fight off infections and diseases. T-cell lymphoma is a type of blood cancer that affects specific immune cells called T-lymphocytes.

T-cell lymphoma starts in lymphoid tissues, which include the lymph nodes, spleen, tonsils, and digestive, or gastrointestinal, tract.

It is a relatively rare disease, accounting for less than 15% of non-Hodgkin lymphomas in the United States.

This article discusses the symptoms, causes, types, diagnosis, and treatment of T-cell lymphoma and the outlook for people with this disease.

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.

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.

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:

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.

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

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

These areas include:

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

People with CTCL may initially notice itchy, dry patches that can appear red or purple, depending on a persons 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.

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.

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.

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.

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 6699% 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 persons 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:

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

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:

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

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.

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:

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:

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 persons 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 24 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.

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T-cell lymphoma: Symptoms, pictures, and treatment - Medical News Today