Peripheral T-cell lymphoma is a lymphoma of the T-cells which are circulating within the lymphatic system after they have left the Thymus. The Thymus is the organ just behind the breast bone where T-cells normally develop and mature. That is how they get the name "T"-cells. B cells on the other hand develop and mature in the bone marrow. There are other types of T-cell lymphoma as well, such as cutaneous t-cell lymphoma which arises in the skin.
Some subtypes of Peripheral T-cell Lymphoma are rare in Western countries but more common in the far east. This is due in part the the prevalence of the HTLV-1 (Human T-cell Lymphotropic Virus) which is considered a risk factor for developing lymphoma. It is more prevalent in Asian countries.
Peripheral T-cell lymphoma can be further subdivided into several subtypes. See the classification page for all the currently recognized types. When the specific subtype cannot be determined it is usually referred to as Peripheral T-cell lymphoma Not Otherwise Specified (NOS)
Some studies show that the prognosis for peripheral t-cell lymphoma is less favourable than for similar aggressive b-cell lymphomas, yet other studies shown no difference in prognosis. As an aggressive lymphoma Peripheral T-cell lymphoma usually requires immediate and aggressive treatment.
Peripheral T-cell lymphoma is treated similarly to other aggressive lymphomas. Usually a doxorubicin based chemotherapy regimen will be used. The anti CD20 monoclonal antibody Rituxan is not used because Rituxan targets the CD20 antigen, which is only found on B-cells and not on T-cells.
Perhaps one of the best reviews of the current status of the prognosis and treatment of peripheral T-cell lymphomas is the 2014 review from the Swedish lymphoma registry. This study was very comprehensive and covered 10 years of data on hundreds of patients. It is the most comprehensive review of its kind for peripheral T-cell lymphoma.
Another important treatment is Pralatrexate. The PROPEL clinical trial showed a 29% response rate in patients with relapsed or refractory Peripheral T-cell lymphoma. Click the link below to read the study results.
Additional information about Peripheral T-cell Lymphoma
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