What Is T-Cell Lymphoma?

T-cell lymphoma is a rare type of non-Hodgkin lymphoma (NHL) that affects cells of the immune system called T-cells, which help protect the body from infections. These lymphomas make up about 12% of all non-Hodgkin lymphoma cases. T-cell lymphoma can be found in different parts of the body: in blood, lymph nodes, and the skin, and sometimes, the T-cells can infiltrate other organs. 

What Causes T-cell Lymphoma?

The exact cause of T-cell lymphoma is unclear, which also means it can’t be directly attributed to one single factor, but several factors that might contribute to the diagnosis. In some cases, having autoimmune diseases, a family history of certain cancers, or conditions like celiac disease may also increase the risk of developing T-cell lymphoma.

Some other factors that can increase the risk for T-cell lymphoma are:

• Genetic changes in T-cells
• Exposure to certain chemicals or chronic inflammation
• Infections from bacteria (like staphylococcus) or viruses such as:

  • HTLV-1 (linked to adult T-cell leukemia/lymphoma)

  • EBV (linked to NK/T-cell lymphoma)

What’s the Difference Between Indolent and Aggressive T-Cell Lymphoma? 

Indolent and aggressive T-cell lymphoma refers to how fast the T-cells grow and how they respond to treatment: 

• Indolent lymphomas: grow slowly and are generally resistant to chemotherapy. One example is cutaneous T-cell lymphoma (CTCL), which mainly affects the skin.

• Aggressive lymphomas: grow quickly and often cause symptoms such as fever, night sweats, and weight loss. One example is peripheral T-cell lymphoma (PTCL) which is a common aggressive type.

Who Can be Affected by  T-cell Lymphoma?

T-cell lymphoma mainly occurs in people between the ages of 55 and 74 and is more common in men. The frequency of T-cell lymphoma can vary based on geographic location and ethnicity. For example, adult T-cell lymphoma is more common in Japan and the Caribbean, while Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is more common in North America.

How Does T-cell Lymphoma Develop?

T-cell lymphomas develop when T-cells, which help fight infections, grow uncontrollably. This can happen due to alterations in specific genes that regulate the T-cell’s cycle, altering how long T-cells live, and how many of them are produced. The genes involved in T-cell’s normal function are; cancer-testis genes and B lymphoid tyrosine kinase. Other cell-signaling pathways such as Jak-3/STAT and NOTCH1 are also linked to the development of T-cell lymphoma. 

How Many T-Cell Lymphoma Types Exist? 

Two large groups comprise T-cell lymphomas: 

• T-lymphoblastic lymphoma/leukemia: constitutes approximately 1% of all lymphomas. It’s most common in teens or young adults, and more males have presented it. It initially affects the thymus, an organ in the chest that stores and matures immune cells. The advantage is that this lymphoma type has a good prognosis. 

• Peripheral T-cell lymphomas have a wide variety of subgroups, each with different characteristics and demographics, and some are easier to treat than others. They are called peripheral because they affect other parts of the body outside the lymph nodes and bone marrow. 

• Cutaneous T-cell lymphomas are a variety of subtypes that affect the skin: (mycosis fungoides, Sezary syndrome, and others)

• Adult T-cell leukemia/lymphoma which is caused by infection with a virus called HTLV-1 

• Angioimmunoblastic T-cell lymphoma (AITL): affects a larger group of patients, representing 4% of all lymphomas. It is more common in older adults and tends to involve besides lymph nodes bone marrow, the spleen, or the liver, which can become enlarged. 

• Extranodal natural killer/T-cell lymphoma, nasal type: This rare type is more common in some parts of Asia and South America. It affects the upper airways, including the nose and throat, but it can also invade the skin, digestive tract, and other organs.

• Enteropathy-associated intestinal T-cell lymphoma (EATL) which occurs in some people with celiac disease and affects the intestines.

• Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) also affects the intestines but is not linked to celiac disease.

• Anaplastic large cell lymphoma (ALCL) which constitutes about 2% of lymphomas, divides into primary cutaneous, systemic, and breast implant-associated. 

In conclusion, T-cell lymphoma represents a diverse and complex group of non-Hodgkin lymphomas that can significantly impact a person’s health, with varying subtypes having different presentations and treatment options. Although the precise causes remain uncertain, several risk factors have been associated with its development. Understanding the distinctions between indolent and aggressive forms, as well as the specific types of T-cell lymphoma, is essential for effective management and treatment. 

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Sources: 

• https://www.ncbi.nlm.nih.gov/books/NBK564354/ 
• https://www.cancer.org/cancer/types/non-hodgkin-lymphoma/about/t-cell-lymphoma.html