Srimanta Chandra Misra*, Gaetano Lucania, Valentina Guarino and Alberto Santagostino
Peripheral T-Cell Lymphomas (PTCL) are an uncommon and heterogeneous group of disorders arising from the innate and adaptive immune system. This review deals with the basis and major revisions to the current PTCL classification. Each entity in the 2016 classification has been reviewed on the basis of cell origin, genetic landscape and recent therapeutic options. The objective of this study was to conduct a review of the normal immune system, signalling pathways and tumor microenvironment in order to understand the heterogeneity of certain entities as well as to uncover the potential therapeutics. A brief evaluation of a normal immune system, implication of the JAK-STAT pathway and tumor microenvironment was performed to explain the heterogeneity of PTCL. Attempts were also made to optimize current standard and personalized management approaches. Fulfilling the current unmet needs in PTCL require optimization of the intensity and number of courses of chemotherapy in first-line treatment, choosing the right strategy of intensification such as ASCT versus improved HSCT and lastly, tailoring the salvage treatment within the currently available options including HSCT, chemo-immunotherapy and targeted therapy. Further knowledge would pave a better future to better manage PTCL.
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Journal of Blood & Lymph received 443 citations as per Google Scholar report
