Peripheral T-cell lymphoma not otherwise specified


Peripheral T-cell lymphoma not otherwise specified, is a subtype of peripheral T-cell lymphoma. Peripheral T-cell lymphoma is defined as a diverse group of aggressive lymphomas that develop from mature-stage white blood cells called T-cells and natural killer cells . PTCL is a type of non-Hodgkin's lymphoma. NHL affects two particular types of white blood cells: B-cells and T-cells. PTCL specifically affects T-cells, and results when T-cells develop and grow abnormally. About 30% of PTCL-NOS cases exhibit malignant T cells that are infected with the Epstein-Barr virus. When associated with EBV, PTCL-NOS is classified as one of the Epstein-Barr virus-associated lymphoproliferative diseases but the relationship of EBV to the development and progression of Epstein-Barr virus-associated PTCL-NOS is unclear.
PTCL-NOS, the most common subtype of PTCL, is aggressive and predominantly nodal. There are two morphologic variants: the T-zone lymphoma variant and the lymphoepithelioid cell variant.
Currently PTCL is treated similarly to B-cell lymphomas. However, in recent years, scientists have developed techniques to better recognize the different types of lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and therapies are being developed that specifically target these types of lymphoma. Currently, however, there are no therapies approved by the US Food and Drug Administration specifically for PTCL. Anthracycline-containing chemotherapy regimens are commonly offered as the initial therapy. Some patients may receive a stem cell transplant. Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation.
Pralatrexate and cerdulatinib are some of the compounds currently under investigations for the treatment of PTC.