title: Genetic alterations in systemic nodal and extranodal non-cutaneous lymphomas derived from mature T cells and natural killer cells.
abstract: Mature (peripheral) T-cell and natural killer (NK)-cell lymphomas comprise a series of rather different neoplasms. Based on morphologic, immunophenotypic, genetic, and clinical data, the World Health Organization classification recognizes more than 20 entities or provisional entities. The variable clinical presentations, the objective recognition and pathological stratification, the difficulties regarding treatment, and the hardly predictable response to therapy indicate that the management of these entities requires novel tools. In contrast to B-cell lymphomas or precursor T-cell neoplasms, few recurrent translocations have been identified so far in T-cell non-Hodgkin's and NK-cell lymphomas. Additionally, some of the entities recognized by the World Health Organization classification are very rare and very scarce molecular data are available for T-cell lymphomas. Here, we have reviewed published reports focusing on the genetic lesions and gene expression profiling underlying systemic nodal and extranodal non-cutaneous mature T-cell and NK-cell lymphomas. We also provide a summary of new agents in clinical development and outline some future directions.
authors.affiliation: Institute of Oncology Research, Bellinzona, Switzerland.
authors.forename: Michela
authors.initials: M
authors.lastname: Boi
authors.forename: Anastasios
authors.initials: A
authors.lastname: Stathis
authors.forename: Emanuele
authors.initials: E
authors.lastname: Zucca
authors.forename: Giorgio
authors.initials: G
authors.lastname: Inghirami
authors.forename: Francesco
authors.initials: F
authors.lastname: Bertoni
mesh_terms: D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology*; D008223:Lymphoma / Q000235:genetics* / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000235:genetics; D013601:T-Lymphocytes / Q000473:pathology*