نظرة عامة
Peripheral T-cell lymphoma (PTCL) is a fast-growing type of lymphoma. Lymphoma is a cancer that affects the lymphocytes, which are a type of white blood cell. The white blood cells are part of the body's germ-fighting immune system.
There are many types of lymphoma. Lymphoma types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. PTCL is a type of non-Hodgkin lymphoma.
PTCL gets its name from the kind of cell that makes up this cancer. It starts in white blood cells called mature T cells, also known as T lymphocytes. The T cells undergo changes and turn into cancer cells. A cell's DNA holds the instructions that tell the cell what to do and when to die. Because of these DNA changes, the cells stop dying. They usually multiply and form a mass. PTCL typically affects lymph nodes but can spread to any organ, including the liver, spleen, skin, bone marrow and digestive tract, also called the GI tract.
PTCL is not one condition. There are many variations called subtypes that fall under the PTCL umbrella. Similar to other types of lymphoma, PTCL undergoes specific DNA changes that help it hide from the immune system.
Treatment depends on the PTCL subtype and may include chemotherapy, radiation therapy, targeted therapy, immunotherapy, clinical trials and bone marrow transplant, also called bone marrow stem cell transplant.
Types
There are many subtypes of peripheral T-cell lymphoma (PTCL). These include but are not limited to:
- Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS): PTCL-NOS is the most common of the PTCL subtypes. This cancer starts in specialized white blood cells called mature T cells or T lymphocytes. The words "not otherwise specified" mean that the cells look like T-cell lymphoma under a microscope but do not match the specific features of other PTCL subtypes. PTCL-NOS occurs in men more often than in women. It also typically affects adults who are middle-aged and older.
- Adult T-cell leukemia-lymphoma (ATL or ATLL): This cancer starts in mature T cells or T lymphocytes. It is associated with human T-lymphotropic virus 1 (HTLV-1) infection. It can show up as cancer cells in the blood, making it more "leukemia-like." Or it can occur as clusters of cells known as masses in lymph nodes or organs. Sometimes both features are present. The condition occurs in men more often than in women and typically affects adults in their mid-50s.
- Anaplastic large cell lymphoma (ALCL): ALCL is a cancer that starts in mature T cells or T lymphocytes that express a protein called CD30. CD30 helps cancer cells hide from the immune system. ALCL has several of its own subtypes that affect various age groups depending on the subtype. Human immunodeficiency virus (HIV) may be linked to ALCL.
- Angioimmunoblastic T-cell lymphoma (AITL): AITL is a cancer of mature T cells or T lymphocytes that comes from a T cell subtype called a T-follicular helper (TFH). TFHs help your body’s white blood cells, called B cells or B lymphocytes, fight germs and viruses. AITL is sometimes called nodal TFH cell lymphoma. It typically affects older adults.
- Enteropathy-associated T-cell lymphoma (EATL) type 1: EATL type 1 is a rare cancer. It usually starts in lymphocytes in the lining of the small intestine. It is linked to Celiac disease. It occurs in men more often than in women and typically affects older adults.
- Extranodal natural killer T-cell lymphoma (ENK-TCL): ENK-TCL is a rare cancer. It usually is associated with an Epstein-Barr virus (EBV) infection. It is called extranodal, as it often begins in tissue outside the lymph nodes. ENK-TCL usually starts inside the nose or throat but also can occur on the skin, on testicles or in the body's digestive tract, also known as the GI tract. ENK-TCL often damages blood vessels and nearby tissue. It occurs in men more than in women and usually affects those in their late 40s to early 50s.
- Hepatosplenic T-cell lymphoma (HSTCL): HSTCL is a rare cancer. It starts in white blood cells called mature T cells or T lymphocytes. The cancer cells often grow in the spleen, liver and bone marrow. Most people find out they have HSTCL once it is more advanced and has spread to other areas of the body. This cancer most often affects men in their 30s. But it also can affect women, children and older adults.
- T-cell prolymphocytic leukemia (T-PLL): T-PLL is a rare cancer that starts in the T-lymphocytes. Known for multiplying quickly, it may appear in the blood, bone marrow, lymph nodes and spleen and on skin. This cancer usually occurs in men more than in women and typically affects older adults.
Diffuse large B-cell lymphoma (DLBCL) is the most common form of non-Hodgkin lymphoma in adults and may be confused with PTCL. DLBCL involves B cells, another type of immune system cell. People who have lymphomas involving B cells often have treatment plans that differ from those that treat PTCL
الأعراض
Symptoms of peripheral T-cell lymphoma (PTCL) vary by subtype. They may include:
- Feeling very tired, known as fatigue.
- Feeling ill.
- Swollen lymph nodes.
- Decreased appetite.
- Weight loss without trying.
- Constant fever.
- Drenching night sweats.
- Swelling in the legs or neck.
- Nausea, bloating or diarrhea.
- Itchy skin.
- Rash.
When to see a doctor
Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you or are not going away.
الأسباب
Peripheral T-cell lymphoma (PTCL) typically forms when germ-fighting white blood cells get changes in their DNA that turn them into cancer cells. The kind of white blood cells involved in PTCL are mature T cells, also called T lymphocytes. A cell's DNA holds the instructions that tell the cell what to do and when to die. Because of these DNA changes, the cells stop dying. Instead, they multiply and spread. Exactly why these DNA changes happen is not known.
Many types of PTCL usually begin in the lymph nodes and can spread to other organs including the liver, spleen, skin, GI tract and bone marrow.
Some PTCL subtypes also may be affected by a weakened immune system, Celiac disease, and infection with viruses such as Epstein-Barr virus (EBV) and human immunodeficiency virus (HIV).
عوامل الخطورة
Some peripheral T-cell lymphoma (PTCL) subtypes tend to affect men more than they affect women. Other risk factors may include:
- Weakened immune system. A weakened immune system might be linked with a higher risk of cancer. People with weakened immune systems include those taking medicine to control the immune system, such as after an organ transplant. Some health conditions, such as HIV infection, also can weaken the immune system.
- Celiac disease is linked to the PTCL subtype enteropathy-associated T-cell lymphoma (EATL).
- Epstein-Barr virus (EBV) infection is linked to the PTCL subtype extranodal natural killer T-cell lymphoma (ENK-TCL).
- Human immunodeficiency virus (HIV) may be linked to the PTCL subtype anaplastic large cell lymphoma (ALCL).
There is no known way to prevent PTCL.
المضاعفات
Peripheral T-cell lymphoma (PTCL) may cause complications as the cancer grows and spreads to other areas of the body. Complications vary by subtype and may include:
- Swollen lymph nodes: PTCL can cause enlarged lymph nodes that may press on nearby organs.
- Tumor lysis syndrome. This serious condition can happen when cancer cells break down quickly after treatment. When the cells die, they release substances into the bloodstream that can overwhelm the body. This can lead to issues with the kidneys and other organs. Medicines may be given to treat or prevent harmful side effects.