Peripheral T-cell lymphoma

Lymphomas are a type of blood cancer that affect the lymphatic system, and peripheral T-cell lymphoma (PTCL) is a very rare type of lymphoma. Since blood cancers can metastasize or spread throughout the body, they are particularly severe and aggressive. Therefore, a fast diagnosis and extensive treatment plan are necessary for a positive prognosis.

Definition & Facts

All types of lymphomas are caused by uncontrollably growing lymphocytes which are a form of white blood cell. They can be divided into Hodgkin's lymphoma and non-Hodgkin's lymphoma based on the type of white blood cell that is affected by the cancer. Peripheral T-cell lymphomas occur when T cell lymphocytes become cancerous; they are a form of non-Hodgkin's lymphoma.

PTCLs differ from other types of T-cell lymphoma because they are very widespread and affect mature cells, and this causes the cancer to spread unusually rapidly. PTCL can further be categorized into many other subsets, including anaplastic large-cell lymphoma, angioimmunoblastic T-cell lymphoma, peripheral T-cell lymphoma not otherwise specified, cutaneous T-cell lymphomas, and enteropathy-associated lymphoma.

Symptoms & Complaints

However, over time, symptoms begin to worsen as the lymphoma expands. Patients may suffer from fatigue or low red blood cell counts if the PTCL is in the bone marrow where it can affect blood cell counts. Other generalized symptoms include unexplained weight loss, night sweats, and a fever.

Depending on the particular location of the PTCL, a patient will also experience other specialized symptoms. A PTCL in the gastrointestinal tract can cause feelings of fullness or pressure and a bloated or swollen abdomen.

PTCLs that are affecting the lungs or nasal area may make it difficult to breathe, cause a sensation of chest pain or heaviness, and result in a chronic cough. Since PTCLs grow at such a fast rate, they may be in multiple organ systems at once, causing all of these symptoms to occur simultaneously.

Causes

All types of cancer are caused by cells in the body multiplying at such an abnormal rate that they impair bodily functions and create tumors. Like all other types of cancer, there are many factors that can cause a cell to become damaged and cancerous, and PTCL has been linked to a variety of genetic factors and environmental factors.

PTCLs most commonly occur in men who are over the age of 60, but it may also develop in people of any other age and gender. People are more likely to get a PTCL if a family member has had one before, so there does seem to be some sort of genetic component that causes PTCLs to develop.

Since it is caused by cancerous immune cells, PTCL is more common among people with autoimmune diseases. Certain viruses can also increase a person's likelihood of getting a PTCL. The Epstein-Barr virus and the human T-cell lymphotropic virus type 1 can cause PTCL after a person is infected with one of these viruses through sexual contact, saliva transfer, or contact with infected blood. Other items that may cause PTCLs are exposure to certain pesticides, fertilizers, or other carcinogenic items.

Diagnosis & Tests

A diagnosis is the first step to getting the treatment needed for a PTCL, and a doctor typically starts seeking a PTCL diagnosis after a physical examination reveals that a patient has some of the basic symptoms of a PTCL. Actually confirming the diagnosis requires several different types of tests.

Since PTCLs are most commonly symptomatic in that the lymph node is enlarged, a biopsy of the lymph node is very helpful. A biopsy will require removal of cells from the swollen node, and then they can be examined in a lab. Once the presence of cancerous cells are confirmed, more tests will be needed to determine the precise type of lymphoma and see how far it has spread throughout the body.

Blood tests, imaging tests such as X-rays or MRIs, and bone marrow samples are all commonly used tests. These tests will be used to diagnose the stage of the PTCL. Stage 1 means that just one area of lymph nodes is affected, while Stage 4 is the most severe stage, and it means that the cancerous cells have spread throughout the body and are growing in other organs.

Treatment & Therapy

Treatment for a PTCL will focus on removing the cancerous cells without harming too many healthy cells. Depending on the specific type and stage of PTCL, a doctor may recommend chemotherapy, radiation, steroid treatment, or surgery to get rid of the lymphoma. The most commonly used type of chemotherapy for a non-Hodgkin's lymphoma is the CHOP method, which uses cyclophosphamide, hydroxydaunomycin, oncovin, and prednisone to shrink the cancer.

If the patient has a good initial response to chemotherapy, a stem cell transplant may be done to boost stem cell levels used to create blood cells because the chemotherapy can kill stem cells. Stem cell transplants help to create new, cancer-free blood cells. If the PTCL is confined to one small area, radiation may be used to destroy cancer cells in a targeted area. There is a very high risk of relapse associated with a PTCL, so regular checkups and scans will be required once a patient goes into remission.

Prevention & Prophylaxis

Avoiding contact with carcinogenic chemicals, including certain types of dyes, solvents, fertilizers, and pollutants, will also reduce risks. One should get regular checkups with one's doctor to ensure that he or she has a better prognosis from early detection. An early diagnosis may help prevent metastasis.