Types of PTCL

PTCLs are sub-classified into various specific diseases based on distinct clinical and/or pathological patterns.

Peripheral T-Cell Lymphoma not otherwise specified (PTCL-NOS) comprises a group of diseases that do not fit into any of the other subtypes of PTCL. PTCL-NOS is the most common subtype, making up about one quarter of all diagnosed PTCLs. It is also the most common of all the T-cell lymphomas. The term PTCL can be confusing as it can refer to the entire spectrum of mature T-cell lymphomas or sometimes to this specific subtype, PTCL-NOS, only. Although most patients with PTCL-NOS present with lymph node involvement, sites outside the lymph nodes, such as the liver, bone marrow, gastrointestinal tract and skin, may also be involved. This group of PTCLs is considered aggressive and requires standard combination chemotherapy upon diagnosis.

Anaplastic Large-Cell Lymphoma (ALCL) is a rare type of aggressive T-cell lymphoma comprising only 3 percent of all lymphomas in adults (about 15 percent to 20 percent of all PTCLs) and between 10 percent and 30 percent of all lymphomas in children. ALCL can appear in the skin or in other organs throughout the body (systemic ALCL). Prognosis and treatment are different for each subtype.

Angioimmunoblastic T-Cell Lymphoma (AITL) is an often fast-growing T-cell lymphoma that accounts for between 1 percent and 2 percent of all NHL cases (about 15 percent to 20 percent of all PTCLs) in the United States. This type of lymphoma often responds to milder therapies, such as steroids, although it often progresses and requires chemotherapy and other medications. In advanced cases, bone marrow transplantation may be used. 

Enteropathy-Type T-Cell Lymphoma is an extremely rare subtype that appears in the intestines and is strongly associated with celiac disease. As with other rare cancers, patients should discuss treatment options with their medical team.

Nasal NK/T-Cell Lymphoma involves natural killer (NK) cells, which have specific jobs in the normal immune system. NK cells are closely related to T-cells and often have features that overlap with normal T-cells. If they become a cancer, they are called an NK or NK/T-cell lymphoma and are grouped with other forms of PTCL. Although this fast-growing lymphoma is very rare in the United States, it is more common in Asia and parts of Latin America, leading researchers suspect that some ethnic groups may be more prone to this cancer. This type of lymphoma is associated with the Epstein-Barr virus and most often involves the nasal area, trachea, gastrointestinal tract or skin. As with other rare cancers, patients should consult with their medical team for treatment options and the availability of clinical trials.

Hepatosplenic Gamma-Delta T-Cell Lymphoma is an extremely rare and aggressive disease that starts in the liver or spleen. As with other rare cancers, patients should discuss treatment options with their medical team.

Cutaneous T-cell Lymphomas (CTCL) are a group of lymphomas that originate in the skin. CTCLs are a subset of PTCL as they are lymphomas of mature T-cells. However, these lymphomas are generally less aggressive, have a different prognosis, and have different treatment approaches than the aggressive PTCLs. Mycosis Fungoides is the most common type of cutaneous T-cell lymphoma. It is generally a slow-growing cancer that starts in the skin, appearing as a scaly, red rash in areas of the body that are not usually exposed to the sun. Sézary Syndrome is an advanced, variant form of mycosis fungoides, and affects both the skin and the peripheral blood. It can cause widespread itching, reddening and peeling of the skin as well as skin tumors.