T-cell lymphoma

Descripción general

T-cell lymphomas are a type of lymphoma. Lymphoma is cancer that affects the lymphatic system. The lymphatic system is made up of organs, glands, tubelike vessels and clusters of cells called lymph nodes. It's part of the body's germ-fighting immune system. T-cell lymphomas can affect lymph nodes, blood, bone marrow, and other tissues throughout the body, such as in the gastrointestinal system, liver, nasal cavity and skin.

There are many types of lymphoma. Lymphoma types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. T-cell lymphomas are a type of non-Hodgkin lymphoma. There are many subtypes of T-cell lymphomas.

T-cell lymphomas get their name from the kind of cell that makes up these cancers. These cancers start in germ-fighting white blood cells called T cells. T cells also are called T lymphocytes.

Treatment for T-cell lymphomas may include a "watch and wait" approach, chemotherapy, immunotherapy, radiation therapy, targeted therapy, clinical trials, and bone marrow transplant, also called bone marrow stem cell transplant.

Types

There are many types of T-cell lymphomas, including:

• Adult T-cell leukemia/lymphoma. This type is caused by a long-term infection with human T-lymphotropic virus 1 (HTLV-1). It is mostly seen in parts of Japan, the Caribbean and Central Africa.
• Anaplastic large cell lymphoma (ALCL). This type has two forms: ALK-positive and ALK-negative forms. ALK-positive disease is more common in younger people and usually responds better to therapy. ALCL also can be linked to breast implants. This is known as breast implant-associated anaplastic large cell lymphoma.
• Cutaneous T-cell lymphomas (CTCL). This type affects the skin and is not found in the lymph nodes or elsewhere in the body. Two common forms of CTCL are mycosis fungoides and Sézary syndrome. CTCL usually shows up first as itchy patches or plaques on the skin.
• Extranodal NK/T-cell lymphoma. This type is linked to Epstein-Barr virus (EBV) infection. It often begins in the nasal passages and can cause facial swelling or nosebleeds.
• Hepatosplenic T-cell lymphoma. This type affects the spleen, liver and bone marrow. It is more common in people who have been immunocompromised.
• Intestinal T-cell lymphoma. This type can affect the intestines, lymph nodes, liver, spleen, lungs and bone marrow. The most common form is enteropathy-associated T-cell lymphoma. It can develop in people with celiac disease. Symptoms of intestinal T-cell lymphoma include belly pain and discomfort, fatigue, nausea, vomiting and diarrhea.
• Nodal T-follicular helper cell lymphoma, angioimmunoblastic-type. This type starts in a type of cell called T follicular helper cells. It mostly affects the lymph nodes. People with nodal T-follicular helper cell lymphoma also often have Epstein-Barr virus (EBV) infection. This type used to be called angioimmunoblastic T-cell lymphoma.
• Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). This is a category for T-cell lymphomas that do not fit other groups. PTCL-NOS typically involves the lymph nodes, liver, bone marrow, gastrointestinal tract and skin.

Healthcare professionals may describe T-cell lymphomas as either aggressive or indolent. Aggressive lymphomas grow quickly and may have more symptoms. Indolent lymphomas grow slowly and may not have symptoms at first. Treatments may vary depending on if the lymphoma is aggressive or indolent. Some indolent lymphomas can transform into aggressive lymphomas.

Síntomas

Symptoms of T-cell lymphomas vary depending on the type and what part of the body is affected. Some people may not experience symptoms. When symptoms happen, they may include:

• Swollen lymph nodes in the neck, armpit and groin.
• Fever.
• Drenching night sweats.
• Fatigue.
• Weight loss without trying.
• Loss of appetite.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

Causas

It's not clear what causes T-cell lymphomas. Cancer happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.

T-cell lymphomas get their names from the kind of cell that makes up these cancers. These cancers start in germ-fighting white blood cells called T cells. T cells also are called T lymphocytes.

Factores de riesgo

Factors that can increase the risk of T-cell lymphomas include:

• A family history of lymphoma. Having a blood relative, such as a parent or sibling, with lymphoma may increase your risk of T-cell lymphomas.
• A weakened immune system or an autoimmune condition. Having a condition that affects the immune system can increase the risk of T-cell lymphomas. These may include irritable bowel disease and celiac disease. The immune system also may be weakened after organ transplant.
• Some infections. Some infections have been linked to types of T-cell lymphomas. These include human lymphotropic virus type 1 (HTLV-1) and Epstein-Barr virus (EBV).

Complicaciones

A complication of T-cell lymphomas is the risk of the cancer coming back after treatment, called a relapse. Sometimes the relapse happens years later. Some people may have multiple relapses. People who relapse have a worse prognosis.