What are the other Names for this Condition? (Also known as/Synonyms)

• PTCL-NOS
• T-Cell NHL
• T-Cell Non-Hodgkin Lymphoma (NHL)

What is Peripheral T-Cell Lymphoma? (Definition/Background Information)

• Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL-NOS), is a type of nodal or soft tissue lymphoma. Nodal lymphomas are restricted to lymph nodes, and generally do not enter the blood stream until later stages of the disease
• Lymphoma is a type of blood cancer stemming from uncontrollably dividing lymphocytes (type of white blood cells). There are two types of lymphomas:
  • Hodgkin Lymphoma
  • Non-Hodgkin Lymphoma
• PTCL-NOS is a type of non-Hodgkin lymphoma. They are differentiated by detecting what proteins are expressed on the surface of the cancer cells, and by the way they look under a microscope
• Lymphocytes are a class of white blood cells that are part of the lymphatic system. Lymphocytes are made in bone marrow, and can develop into either B-cells or T-cells. T-cells mature in the thymus; PTCL arises from cancerous T-cells
• Normally, lymphocytes help generate an immune response to infections. These cells can recognize a wide variety of foreign invaders. They can also remember them and respond accordingly, if the body is infected with the same bacteria/virus ever again
• The lymphatic system is responsible for immunity to diseases. The organs of the lymphatic system are lymph nodes, bone marrow, the spleen, thymus, and tonsils. T-cells can travel from the thymus to other parts of the lymphatic system
• T-cells are responsible for recognizing cells that are altered or infected, and it includes attempting to kill cells that are cancerous too

Who gets Peripheral T-Cell Lymphoma? (Age and Sex Distribution)

• Peripheral T-Cell Lymphoma is relatively uncommon and only accounts for 15% of non-Hodgkin lymphoma cases (in the US). PTCL-NOS is the most common subtype of PTCL and accounts for 25% of the cases
• Adults aged 55-60 years old are at the highest risk for PTCL-NOS
• Men are twice as likely as women, to develop the condition

What are the Risk Factors for Peripheral T-Cell Lymphoma? (Predisposing Factors)

There are very few risk factors associated with Peripheral T-Cell Lymphoma, NOS and these include:

• A diagnosis of Epstein-Barr virus (virus causing mononucleosis) has been made in 30% of PTCL cases, but the connection between the two, is unknown
• Exposure to chemicals, such as pesticides and fertilizers, as well as smoking and diet, has been suggested as links to other forms of non-Hodgkin lymphoma.
• No other links to inherited disorders or immunological disorders have been made at this time

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Peripheral T-Cell Lymphoma? (Etiology)

• The exact cause of Peripheral T-Cell Lymphoma, NOS is not completely understood
• PTCL arises from mutations in the DNA of a single T-cell, which causes it to divide and make more cells uncontrollably

What are the Signs and Symptoms of Peripheral T-Cell Lymphoma?

The signs and symptoms of Peripheral T-Cell Lymphoma are:

• Non-painful swelling of lymph nodes in the neck, armpits, or groin
• Loss of appetite
• Fatigue
• Excessive night sweats
• High temperatures
• Weight loss
• Anemia (low red blood cell count)

How is Peripheral T-Cell Lymphoma Diagnosed?

It often takes multiple tests to diagnose lymphoma. It is critical that the specific sub-type of lymphoma be diagnosed correctly, in order to create a proper treatment plan. Peripheral T-Cell Lymphoma, NOS diagnosis involves:

• A physical examination by a doctor can help determine, if symptoms like lymph node swelling, are likely caused by an infection, or if there is a risk of lymphoma
• Biopsies of enlarged lymph nodes are taken and examined in a lab, to determine if the cells are malignant or benign. The biopsies may be performed under general or local anesthesia. Normally, the entire lymph node is removed to help determine the sub-type of lymphoma.
• Bone marrow biopsies are performed, to determine if the bone marrow is involved
• Blood tests
• Lumbar puncture
• X-rays
• Whole body CT-PET scan - to determine how far the lymphoma has spread, by checking the size and metabolic rate (a reflection of uncontrolled growth) of lymph nodes, throughout the body
• Brain MRIs are used if neurologic symptoms are present, to determine if cancer has spread to the brain, or to the tissue that covers the brain

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Peripheral T-Cell Lymphoma?

The possible complications from Peripheral T-Cell Lymphoma, NOS include:

• PTCL-NOS can spread from the lymph nodes to other parts of the body
• Treatments can cause secondary health problems, such as secondary forms of cancer and heart disease
• There is a high risk of relapse and a low percent of long-term survival

How is Peripheral T-Cell Lymphoma Treated?

Once a definitive diagnosis of Peripheral T-Cell Lymphoma, NOS has been made, staging is used to describe how far the cancer has spread. The stage can describe:

• How many lymph nodes are affected
• Their locations in the body
• And, if other organs are being affected

Staging is important because different treatment regimens are necessary, depending on the progression of the lymphoma.

• Stage 1: Only one group of lymph nodes in one location is affected
• Stage 2: Lymph nodes in multiple locations that are all above or below the diaphragm are affected
• Stage 3: Lymph nodes in multiple locations, both above and below the diaphragm are affected
• Stage 4:  Lymph nodes located throughout the body, the bone marrow, and other organs are affected

A combination of treatments is used to most effectively treat this cancer. The aggressiveness of the treatment is determined by the disease stage.

Surgery: After initial biopsies to diagnose PTCL-NOS, surgery is a very uncommon treatment option, because lymphomas are systemic and have already spread to more than one lymph node in most patients. Removing all affected lymph nodes would be extremely difficult.

Chemotherapy: This approach uses a combination of drugs to kill the cancerous cells and can be used for patients, at all stages of PTCL.

• Commonly, the drug combination CHOP is used first, which includes doxorubicin, vincristine, cyclophosphamide, and prednisolone
• There can be severe side effects including fatigue, nausea, hair loss, anemia, high risk of infection, and drug-specific reactions
• Many T-cell lymphomas can be resistant to chemotherapy. It can also damage healthy cells
• Chemotherapy can be administered as a pill, liquid, shot, or intravenously

Radiation: Radiation therapy is the use of high-energy radiation waves, to kill cancer cells by destroying their DNA.

• This treatment modality is generally used for early stage lymphomas. It is most commonly used in combination with chemotherapy
• The radiation may be administered by a machine placed outside the body, or by putting a radioactive material inside the body
• The side effects of radiation therapy include nausea, vomiting, fatigue, pain, risk of (further) cancer later in life, and a risk of heart disease
• Radiation can damage healthy cells in addition to cancer cells, causing further complications

Stem cell support: In combination with chemotherapy, blood stem cells can be taken from a patient before treatment and administered to them, later during the treatment, in order to help restore blood cells killed by chemotherapy. This treatment can have severe side effects; hence, it is not administered to all patients.

Clinical trials: There are some new treatment options, currently on clinical trials that can be considered for some patients, depending on their risk factors.

How can Peripheral T-Cell Lymphoma be Prevented?

• Currently, there are no definitive ways to prevent Peripheral T-Cell Lymphoma
• However, healthy diet and exercise, as well as avoidance of unnecessary exposure to chemicals, may help decrease its risk
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the tumor, due to its metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Peripheral T-Cell Lymphoma? (Outcomes/Resolutions)

• Peripheral T-Cell Lymphoma, NOS is an aggressive form of T-cell Lymphoma with a high risk of relapse
• The overall survival rate for patients with PTCL-NOS, over a 5-year period is 30%. This drops to 5%, at 10-15 years after diagnosis

Additional and Relevant Useful Information for Peripheral T-Cell Lymphoma:

• T-cell lymphomas are less common than B-cell lymphomas
• PTCL-NOS occurs more commonly in the Far East (Asian countries), than the western countries
• Peripheral T-Cell Lymphoma, Not Otherwise Specified - indicates a general category, which means that there is no specific subtype of T-cell lymphoma that applies to the tumor