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Peripheral T-cell lymphoma (PTCL) is the name given to a group of rare forms of non-Hodgkin lymphoma (NHL). PTCL is a malignant condition (cancer) affecting a type of white blood cells called lymphocytes.
Peripheral T-cell lymphoma is a type of non-Hodgkin lymphoma (NHL) which makes up less than 1 in 10 cases of adult NHL. There are several different sub-types of PTCL based on features of the T cells. Lymphoma is a malignant condition (cancer) affecting a type of white blood cells called lymphocytes. Unlike leukaemia, in lymphoma, the cancer cells are found in organs and tissues of the lymphatic system. The lymphatic system is a network of fine vessels, glands and channels which occur throughout the body. It also conveys nutrients and cells, and is responsible for draining fluid and waste products away from tissues, and into the blood stream to be processed. The lymphatic system is an important part of the immune system and is made up of lymph nodes and vessels and of collections of lymphocytes in other tissues.
PTCL is a high-grade form of NHL, this means that it develops quickly and, if not treated, it grows quickly. Peripheral refers to where in the body it most commonly appears and T cell describes the type of lymphocyte which is affected.
There are 20 sub-types but about 60% of all PTCL is made up of four sub-types;
PTCL, NOS includes all cases of PTCL which don’t fit one of the other sub-types. ALK+ or ALK- refers to the results of a particular laboratory test. They are classified separately because treatments and likely response are different.
In most cases of PTCL, there is no obvious cause. It is important to understand that:
The symptoms of PTCL vary according to the subtype and which part of the body is affected. Often PTCL affects lymph nodes leading to a lump in the neck, armpit or groin or stomach. These are usually, but not always, painless and are often described as feeling “rubbery”. Other common symptoms include:
The most important test for diagnosis of PTCL is a biopsy of affected tissue. This may mean removing a whole abnormal lymph node (excision biopsy) or just a sample of tissue.
Other tests which may be done include:
Staging is the use of test results to show how widely the lymphoma has spread. There are four stages in the standard system used:
Stages 1-2 are known as early-stage disease and stages 3-4 are known as advanced-stage disease.
This stage description may be modified by adding the letters A, B, E or S:
Almost all patients with PTCL will start treatment soon after diagnosis, unfortunately, PTCL is very difficult to treat and often does not respond well to chemotherapy. A number of new types of treatment are being evaluated for treatment of PTCL.
PTCL is curable in some cases but more often patients will have a remission (a period when the lymphoma is well controlled) but it will come back, this is known as a relapse. A relapse may respond to further treatment. Some sub-types respond better than others and you should be given detailed advice on what you can expect to happen.
The main ways in which PTCL is treated are:
Chemotherapy is the use of cell-killing drugs. These kill the cancer cells and/or stop them from dividing. Chemotherapy is usually given in blocks or ‘cycles’ of treatment. One cycle of treatment will consist of a series of doses of chemotherapy followed by a break for the healthy cells to recover.
Chemotherapy is normally given as a combination of drugs, which will usually include steroids. Steroids used to treat PTCL are a laboratory-made version of chemicals naturally made by the body. They are very different from the type of steroids sometimes misused by body-builders or sportsmen.
The details of your treatment will vary depending on the stage and sub-type of your PTCL and your general fitness. You will be given a chance to discuss treatment options and detailed information on your treatment plan before it starts. The side effects of treatment vary between different types of treatment and different patients. You will be given detailed information about any likely side effects before you start treatment.
We understand going through a blood cancer through journey can be difficult. It may help to talk to a close friend or relative about how you are feeling. Here are some questions that may be useful to ask your doctor.