Peripheral T-cell lymphoma is a rare and aggressive cancer affecting immune T cells. It is divided into two types by the World Health Organization and can progress rapidly, invading various parts of the body. Treatment includes chemotherapy, radiation, and other drugs, and clinical trials are being conducted to improve survival rates.
Peripheral T-cell lymphoma (PTCL) is a cancer of the white blood cells that affects immune T cells, which circulate throughout the body through the lymphatic system after being produced in the thymus gland. The disease is non-Hodgkin’s lymphoma, which means it is similar to cancers classified as Hodgkin’s, but lack the presence of a certain distinguishing cell, the Reed-Sternberg cell. It is a relatively rare disease, accounting for only a small percentage of all non-Hodgkin lymphomas in the United States. Statistical data is also believed to be similar in other westernized countries. The cancer is considered aggressive and treatment for the disease is usually started as early as possible to increase the chances of survival.
The World Health Organization (WHO) divides peripheral T-cell lymphoma into two types: precursor and true peripheral PTCL. The designations take into account the genesis of cancerous T cells. For example, the PTCL precursor is thought to be composed primarily of cancerous lymphoblastic cells, the cells that essentially produce and are precursors of immune system lymph. Both types of peripheral T-cell lymphoma are typically diagnosed in people 60 years of age and older and more often in men than in women. An exception to this statistic is a precursor to PTCL, which is more commonly diagnosed in young males adults and adolescents; this disease designation has a higher complete remission rate than other forms of PTCL, nearly 96 percent.
A form of true peripheral T-cell lymphoma, called blastic NK-cell lymphoma, is the rarest and most difficult to treat of all the disease designations. It is very fast growing and can arise anywhere in the body as opposed to other lymphomas that arise in specific places. Because of this, the treatment is more complicated and has a higher risk profile of side effects. Patients diagnosed with the rarest forms of PTCL are often good candidates for clinical trials, where significant progress is being made in survival rates.
PTCL progresses rapidly and often invades the liver, gastrointestinal tract, and bone marrow. One reason peripheral T-cell lymphoma is so aggressive is because it usually infects lymph nodes to a high degree, causing them to spread rapidly. PTCL is treated in much the same way most cancers are treated; with chemotherapy, radiation, and other type-specific drugs. Sometimes, PTCL is successfully treated by doing a bone marrow transplant or supplementing treatment with steroid medications.
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