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Peripheral T cell lymphoma, not otherwise specified : the stuff of genes, dreams and therapies

Agostinelli, C. and Piccaluga, P. P. and Went, P. and Rossi, M. and Gazzola, A. and Righi, S. and Sista, T. and Campidelli, C. and Zinzani, P. L. and Falini, B. and Pileri, S. A.. (2008) Peripheral T cell lymphoma, not otherwise specified : the stuff of genes, dreams and therapies. Journal of clinical pathology, Vol. 61, no. 11. pp. 1160-1167.

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Official URL: http://edoc.unibas.ch/dok/A6007628

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Abstract

Peripheral T cell lymphomas (PTCL) account for about 12% of lymphoid tumours worldwide. Almost half show such morphological and molecular variability as to hamper any further classification, and to justify their inclusion in a waste-basket category termed "not otherwise specified (NOS)". The latter term is used for neoplasms with aggressive presentation, poor response to therapy and dismal prognosis. In contrast to B cell lymphomas, PTCL have been the subject of only a limited number of studies to elucidate their pathobiology and identify novel pharmacological approaches. Herewith, the authors revise the most recent contributions on the subject based on the experience they have gained in the extensive application of microarray technologies. PTCL/NOS are characterised by erratic expression of T cell associated antigens, including CD4 and CD52, which have recently been proposed as targets for ad hoc immunotherapies. PTCL/NOS also show variable Ki-67 marking, with rates <80% heralding a worse prognosis. Gene expression profiling studies have revealed that PTCL/NOS derive from activated T lymphocytes, more often of the CD4+ type, and bear a signature composed of 155 genes and related products that play a pivotal role in cell signalling transduction, proliferation, apoptosis and matrix remodelling. This observation seems to pave the way for the use of innovative drugs such as tyrosine kinase and histone deacetylase inhibitors whose efficacy has been proven in PTCL primary cell cultures. Gene expression profiling also allows better distinction of PTCL/NOS from angioimmunoblastic T cell lymphoma, the latter being characterised by follicular T helper lymphocyte derivation and CXCL13, PD1 and vascular endothelial growth factor expression.
Faculties and Departments:03 Faculty of Medicine > Bereich Querschnittsfächer (Klinik) > Pathologie USB
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Querschnittsfächer (Klinik) > Pathologie USB
UniBasel Contributors:Went, Philip Th.
Item Type:Article, refereed
Article Subtype:Further Journal Contribution
Publisher:British Medical Association
ISSN:0021-9746
Note:Publication type according to Uni Basel Research Database: Journal item
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Last Modified:20 Jun 2014 07:55
Deposited On:20 Jun 2014 07:55

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