Abstract
Based on their own experience and knowledge of the literature, the authors revise the pathobiological characteristics of peripheral T-cell lymphomas (PTCL) by focusing on the most recent data available as far as gene expression profile (GEP) analyses are concerned.
First, GEP studies provided important insight into the histogenesis, molecular pathogenesis, and targeted treatments of different PTCL subtypes. For example, it was clearly shown that angioimmunoblastic T-cell lymphoma (AITL) corresponds to T follicular helper (TFH) lymphocytes and presents consistent deregulation of genes involved in angiogenesis. Noteworthy, targeting some of them, such as VEGF/VEGFR2, may represent an innovative and effective therapeutic strategy. Secondly, it was shown that PTCLs/not otherwise specified (PTCL/NOS) include at least three different subset characterized by specific cellular derivation (T-central memory, T-cytotoxic, and TFH) and possibly different outcome. Besides that, notably, all PTCLs/NOS present with constant deregulation of certain molecules, including the PDGFRA, which represents a suitable therapeutic target in this setting. Finally, both ALK+ and ALK− ALCLs have been shown to be distinct from the other PTCLs, possibly constituting separate entities. Remarkably, the molecular profile of the ALK+ forms largely relies on the activation of ALK and its downstream STAT3, while other tyrosine kinases are probably activated in the ALK− ones.
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Piccaluga, P.P., Pileri, S.A. (2013). Molecular Profiling of Peripheral T-Cell Lymphomas. In: Younes, A., Coiffier, B. (eds) Lymphoma. Current Clinical Oncology, vol 43. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-408-1_3
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