Vol 52, No 4 (2021)
Review article
Published online: 2021-08-31
Treatment of peripheral T-cell lymphomas
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Acta Haematol Pol 2021;52(4):334-339.
Abstract
Peripheral T-cell lymphomas (PTCLs) are rare neoplasms that recently have been the subject of much research into their complex pathophysiology. PTCLs are a heterogeneous group of tumors consisting of nodal and extranodal leukemic and cutaneous neoplasms. PTCLs are associated with complex biology and arduous pathology which is currently being studied. According to this research, the pathophysiology of PTCLs can be divided into intrinsic and extrinsic mechanisms. Among the intrinsic mechanisms, scientists have described JAK-STAT pathway deregulation, as well as different somatic mutations including RB1, PTEN, TP53 and structural changes to the receptors. Also, there are scientific papers that correlate Epstein-Bárr virus or human T-cell lymphotropic virus type 1 infections with the occurrence of the neoplasm. PTCLs are most likely to develop in Asian and African populations. Due to poor clinical outcomes, PTCL treatment is the subject of intense clinical research. As a result of that, new drugs have been approved by the Food and Drug Administration for use among patients with refractory PTCL: pralatrexate, an antifolate drug; romidepsin, belinostat, an inhibitor for histone deacetylase, and brentuximab vedotin, a CD30 antibody. Also, clinical trials with mogamulizumab are being carried out for PTCL treatment. In addition to this, lenalidomide, as a substance that regulates the immune system and has shown antineoplastic effect in several hematological studies, could possibly be considered as treatment.
Keywords: T-cell lymphomaperipheral T-cell lymphomabrentuximab vedotinmogamulizumab
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