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Purpose of Review Peripheral T cell lymphoma (PTCL) is a heterogeneous group of lymphoproliferative neoplasms, with at least 29 distinct entities described in current WHO classification. Using present diagnostic approaches, more than a third of PTCL cases cannot be classified, hence designated as PTCL-not otherwise specified (PTCL-NOS). Herein, we summarize the current genomic findings and their role in the molecular pathogenesis in different PTCL entities. Recent Findings Gene expression profiling (GEP) studies have identified distinct molecular signatures for accurate diagnosis and elucidated oncogenic pathways enriched in major PTCL entities. Furthermore, genomic characterization has identified recurrent somatic mutations and potential therapeutic targets. Further efforts are underway to develop genetically faithful murine models. Summary GEP studies have identified molecular subgroups of PTCL, characterized by distinct genetic and epigenetic alter- ations. Understanding the molecular mechanisms of T cell lymphomagenesis using in vivo model will help to reveal novel therapeutic targets. . . . Keywords PTCL Peripheral Tcell lymphoma-not otherwise specified Angioimmunoblastic Tcell lymphoma Anaplastic large . . cell lymphoma Gene expression profiling Molecular signature Introduction cell lymphoma (ALCL), adult T cell leukemia/lymphoma (ATLL), and extranodal NK/T cell lymphoma of nasal type Peripheral T cell lymphoma (PTCL) is a heterogeneous group (ENKTL) [5].
Current Hematologic Malignancy Reports – Springer Journals
Published: Jun 27, 2018
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