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Peripheral T-cell lymphoma (PTCL) consists of a group of rare and usually aggressive non-Hodgkin lymphomas (NHLs) that develop from mature T-cells.
There are 27 subtypes of PTCL (WHO, 2016), of which the most common 6 subtypes account for >75% of cases. These include PTCL-NOS, anaplastic lymphoma kinase (ALK+/ALK-) anaplastic large cell lymphoma (ALCL), angioimmunoblastic T-cell lymphoma (AITL), NK/T-cell lymphoma, and adult T-cell leukemia/lymphoma (ATLL).
The CHOP-based chemotherapy regimen has remained the standard of treatment for most PTCLs in recent years; however, newer treatments are now available for PTCL, including antibody-drug conjugates such as brentuximab vedotin, and ALK inhibitors are promising for ALK expressing tumors. The use of checkpoint inhibitors remains controversial.