T-Cell Lymphoma – Lymphoma Research Foundation
T-Cell Lymphomas
Common Types of T-cell Lymphoma
Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) refers to a group of diseases that do not fit into any of the other subtypes of PTCL. PTCL-NOS is the most common PTCL subtype, making up about one-quarter of all 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. Albeit most patients with PTCL-NOS are diagnosed with their disease held to the lymph knots, sites outside the lymph knots, such as the liver, bone marrow, gastrointestinal tract, and skin, may also be involved. This group of PTCLs is aggressive and requires combination chemotherapy upon diagnosis. For more information, see the Lymphoma Research Foundation’s (LRF) Peripheral T-Cell Lymphoma Fact Sheet.
Anaplastic large cell lymphoma describes several types of T-cell lymphomas and accounts for approximately twelve percent to fifteen percent of all T-cell lymphomas in adults and inbetween ten percent and thirty percent of all lymphomas in children. It can be divided into three types. There are two systemic (presents in lymph knots or organs) subtypes and one non-systemic type. The systemic subtypes are anaplastic lymphoma kinase (ALK) positive or ALK negative anaplastic large cell lymphoma, depending on the presence or absence of an abnormal form of the ALK protein on the surface of the lymphoma cells. The non systemic type is called primary cutaneous anaplastic large cell lymphoma, and it shows up only on the skin. The systemic types are usually fast-growing, while the skin-only type is usually more slow-growing. For more information, see LRF’s Anaplastic Large Cell Lymphoma Fact Sheet.
Angioimmunoblastic Lymphoma is a fast-growing T-cell lymphoma accounting for fifteen percent to eighteen percent of all T-cell lymphomas in the United States. Initial symptoms often include engorged lymph knots and systemic symptoms such as fever and rash. It is generally treated like other fast-growing T-cell lymphomas, but can be managed with milder therapies in certain circumstances. For more information, see LRF’s Angioimmunoblastic Lymphoma Fact Sheet.
Cutaneous T-cell lymphoma accounts for two percent to three percent of all NHL cases and usually affects adults. The term cutaneous T-cell lymphoma describes a group of typically slow-growing cancers that emerge on, and are most often restrained to, the skin. Mycosis fungoides, which emerges as skin patches or plaques, is the most common type of cutaneous T-cell lymphoma. Less common forms include Sézary syndrome, primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis. For more information, see LRF’s Cutaneous T-Cell Lymphoma Fact Sheet.
Types of T-cell Lymphoma: Relatively Less Common
Adult T-cell Leukemia/Lymphoma (ATLL) is a infrequent form of T-cell lymphoma linked to infection by the human T-cell lymphotropic virus type one (HTLV-1) virus. This virus is commonly found in people from the Caribbean, parts of southern Japan, and central Africa, as well as sporadic cases from around the world. While people usually acquire the virus at birth or during breastfeeding, only about two percent who carry the virus will develop lymphoma. The good majority will remain asymptomatic carriers across their life. This lymphoma can be indolent (slow-growing) or aggressive (fastgrowing). For more information, see LRF’s Adult T-Cell Leukemia/ Lymphoma Fact Sheet.
Blastic NK-cell Lymphoma is a very uncommon cancer, affecting only a few people (usually adults) each year. This lymphoma was previously thought to arise from a T- or NK-cell. However, newer studies indicate that it may arise from another type of white blood cell called a plasma, or dendritic, cell. This lymphoma is fast-growing and can be difficult to treat. It can arise anywhere in the bod. Dark crimson or purple skin lesions are a common feature.
Enteropathy-type T-cell lymphoma iis an enormously uncommon subtype of T-cell lymphoma that emerges in the intestines and is strongly associated with celiac disease.
Hematosplenic gamma-delta T-cell Lymphoma is an utterly infrequent and aggressive disease that starts in the liver or spleen. This lymphoma may occur in those with inflammatory bowel disease who are immunosuppressed (that is, their immune system was suppressed as part of treatment).
Lymphoblastic Lymphoma can show up in both B-cells and T-cells, but is much more common in T-cells, comprising eighty percent of all lymphoblastic lymphomas. This lymphoma is most often diagnosed in children. With intensive chemotherapy, the finish remission rate can be very high.
Nasal NK/T-cell Lymphomas are relatively uncommon in the United States, but common in Asia and parts of Latin America. It is a fast-growing lymphoma that typically originates in the lining of the nose or upper airway. It is treated with radiation and various combinations of chemotherapy.
Treatment-related T-cell lymphomas sometimes show up after solid organ or bone marrow transplantation. The immune system suppression that is required to transplant patients can put them at risk for developing these lymphomas. Treatment-related T-cell lymphomas may require therapy that differs from the standard treatments normally used to treat these conditions.
Patients diagnosed with the infrequent forms of lymphoma should consult their medical team to find promising therapies or clinical trials.
Treatment Options
Because there are so many different types of T-cell lymphoma, treatment varies widely. Standard lymphoma therapies, including chemotherapy, radiation, stem cell transplantation, and surgery, may be effective. Patients diagnosed with the infrequent forms of lymphoma should consult their medical team to find promising therapies or clinical trials.
Treatments aimed at the skin, such as ultraviolet light therapy or electron slat therapy (a type of radiation that does not penetrate to internal organs), are effective for many slow-growing T-cell lymphomas that emerge in the skin. Drugs that have been approved specifically for T-cell lymphomas of the skin include bexarotene (Targretin), denileukin diftitox (Ontak), romidepsin (Istodax), and vorinostat (Zolinza).
A procedure called extracorporeal photopheresis (ECPP) is approved to treat people with mycosis fungoides or Sézary syndrome. For this procedure, blood is eliminated from the patient and treated with ultraviolet light, and with drugs that become active when exposed to ultraviolet light. Once the blood has been treated, it is then returned back into the patient’s figure.
Several agents are approved for the treatment of T-cell lymphomas. Pralatrexate (Folotyn) was approved in two thousand nine for relapsed (recurrence of the disease) or refractory (disease that is resistant to treatment) peripheral T-cell lymphoma. Romidepsin was approved in two thousand nine for the treatment of relapsed or refractory CTCL and in two thousand eleven for the treatment of relapsed or refractory peripheral T-cell lymphoma.
Treatments Under Investigation
Treatment options for the different types of T-cell lymphomas are expanding as fresh treatments are discovered and current treatments are improved. For example, a pilot investigate of sorafenibin is examining the use of biomarkers in relapsed or refractory patients and a phase I/II explore is presently investigating everolimus plus chemotherapy with CHOP in patients who were recently diagnosed with peripheral T-cell lymphomas.
Clinical Trials
Go after Up
Lymphoma survivors should have regular visits with a physician who is familiar with their medical history as well as the treatments they have received.
Some treatments can cause long-term effects or late effects, which can vary based on duration and frequency of treatments, age, gender, and overall health of each patient at the time of treatment. The doctor will check for these effects during follow-up care.
Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be significant for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our fact sheet on survivorship issues.
T-Cell Lymphoma – Lymphoma Research Foundation
T-Cell Lymphomas
Common Types of T-cell Lymphoma
Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) refers to a group of diseases that do not fit into any of the other subtypes of PTCL. PTCL-NOS is the most common PTCL subtype, making up about one-quarter of all 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. Albeit most patients with PTCL-NOS are diagnosed with their disease restricted to the lymph knots, sites outside the lymph knots, such as the liver, bone marrow, gastrointestinal tract, and skin, may also be involved. This group of PTCLs is aggressive and requires combination chemotherapy upon diagnosis. For more information, see the Lymphoma Research Foundation’s (LRF) Peripheral T-Cell Lymphoma Fact Sheet.
Anaplastic large cell lymphoma describes several types of T-cell lymphomas and accounts for approximately twelve percent to fifteen percent of all T-cell lymphomas in adults and inbetween ten percent and thirty percent of all lymphomas in children. It can be divided into three types. There are two systemic (presents in lymph knots or organs) subtypes and one non-systemic type. The systemic subtypes are anaplastic lymphoma kinase (ALK) positive or ALK negative anaplastic large cell lymphoma, depending on the presence or absence of an abnormal form of the ALK protein on the surface of the lymphoma cells. The non systemic type is called primary cutaneous anaplastic large cell lymphoma, and it emerges only on the skin. The systemic types are usually fast-growing, while the skin-only type is usually more slow-growing. For more information, see LRF’s Anaplastic Large Cell Lymphoma Fact Sheet.
Angioimmunoblastic Lymphoma is a fast-growing T-cell lymphoma accounting for fifteen percent to eighteen percent of all T-cell lymphomas in the United States. Initial symptoms often include engorged lymph knots and systemic symptoms such as fever and rash. It is generally treated like other fast-growing T-cell lymphomas, but can be managed with milder therapies in certain circumstances. For more information, see LRF’s Angioimmunoblastic Lymphoma Fact Sheet.
Cutaneous T-cell lymphoma accounts for two percent to three percent of all NHL cases and usually affects adults. The term cutaneous T-cell lymphoma describes a group of typically slow-growing cancers that show up on, and are most often restricted to, the skin. Mycosis fungoides, which shows up as skin patches or plaques, is the most common type of cutaneous T-cell lymphoma. Less common forms include Sézary syndrome, primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis. For more information, see LRF’s Cutaneous T-Cell Lymphoma Fact Sheet.
Types of T-cell Lymphoma: Relatively Less Common
Adult T-cell Leukemia/Lymphoma (ATLL) is a uncommon form of T-cell lymphoma linked to infection by the human T-cell lymphotropic virus type one (HTLV-1) virus. This virus is commonly found in people from the Caribbean, parts of southern Japan, and central Africa, as well as sporadic cases from around the world. While people usually acquire the virus at birth or during breastfeeding, only about two percent who carry the virus will develop lymphoma. The superb majority will remain asymptomatic carriers across their life. This lymphoma can be indolent (slow-growing) or aggressive (fastgrowing). For more information, see LRF’s Adult T-Cell Leukemia/ Lymphoma Fact Sheet.
Blastic NK-cell Lymphoma is a very infrequent cancer, affecting only a few people (usually adults) each year. This lymphoma was previously thought to arise from a T- or NK-cell. However, newer studies indicate that it may arise from another type of white blood cell called a plasma, or dendritic, cell. This lymphoma is fast-growing and can be difficult to treat. It can arise anywhere in the figure. Dark crimson or purple skin lesions are a common feature.
Enteropathy-type T-cell lymphoma iis an utterly infrequent subtype of T-cell lymphoma that shows up in the intestines and is strongly associated with celiac disease.
Hematosplenic gamma-delta T-cell Lymphoma is an enormously uncommon and aggressive disease that starts in the liver or spleen. This lymphoma may occur in those with inflammatory bowel disease who are immunosuppressed (that is, their immune system was suppressed as part of treatment).
Lymphoblastic Lymphoma can emerge in both B-cells and T-cells, but is much more common in T-cells, comprising eighty percent of all lymphoblastic lymphomas. This lymphoma is most often diagnosed in children. With intensive chemotherapy, the accomplish remission rate can be very high.
Nasal NK/T-cell Lymphomas are relatively infrequent in the United States, but common in Asia and parts of Latin America. It is a fast-growing lymphoma that typically originates in the lining of the nose or upper airway. It is treated with radiation and various combinations of chemotherapy.
Treatment-related T-cell lymphomas sometimes emerge after solid organ or bone marrow transplantation. The immune system suppression that is required to transplant patients can put them at risk for developing these lymphomas. Treatment-related T-cell lymphomas may require therapy that differs from the standard treatments normally used to treat these conditions.
Patients diagnosed with the infrequent forms of lymphoma should consult their medical team to find promising therapies or clinical trials.
Treatment Options
Because there are so many different types of T-cell lymphoma, treatment varies widely. Standard lymphoma therapies, including chemotherapy, radiation, stem cell transplantation, and surgery, may be effective. Patients diagnosed with the uncommon forms of lymphoma should consult their medical team to find promising therapies or clinical trials.
Treatments aimed at the skin, such as ultraviolet light therapy or electron slat therapy (a type of radiation that does not penetrate to internal organs), are effective for many slow-growing T-cell lymphomas that show up in the skin. Drugs that have been approved specifically for T-cell lymphomas of the skin include bexarotene (Targretin), denileukin diftitox (Ontak), romidepsin (Istodax), and vorinostat (Zolinza).
A procedure called extracorporeal photopheresis (ECPP) is approved to treat people with mycosis fungoides or Sézary syndrome. For this procedure, blood is eliminated from the patient and treated with ultraviolet light, and with drugs that become active when exposed to ultraviolet light. Once the blood has been treated, it is then returned back into the patient’s bod.
Several agents are approved for the treatment of T-cell lymphomas. Pralatrexate (Folotyn) was approved in two thousand nine for relapsed (recurrence of the disease) or refractory (disease that is resistant to treatment) peripheral T-cell lymphoma. Romidepsin was approved in two thousand nine for the treatment of relapsed or refractory CTCL and in two thousand eleven for the treatment of relapsed or refractory peripheral T-cell lymphoma.
Treatments Under Investigation
Treatment options for the different types of T-cell lymphomas are expanding as fresh treatments are discovered and current treatments are improved. For example, a pilot examine of sorafenibin is examining the use of biomarkers in relapsed or refractory patients and a phase I/II probe is presently investigating everolimus plus chemotherapy with CHOP in patients who were recently diagnosed with peripheral T-cell lymphomas.
Clinical Trials
Go after Up
Lymphoma survivors should have regular visits with a physician who is familiar with their medical history as well as the treatments they have received.
Some treatments can cause long-term effects or late effects, which can vary based on duration and frequency of treatments, age, gender, and overall health of each patient at the time of treatment. The doctor will check for these effects during follow-up care.
Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be significant for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our fact sheet on survivorship issues.