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dc.contributor.authorZambello, Renato
dc.contributor.authorTeramo, Antonella
dc.contributor.authorGattazzo, Cristina
dc.contributor.authorSemenzato, Gianpietro
dc.date.accessioned2014-06-04T09:50:51Z
dc.date.available2014-06-04T09:50:51Z
dc.date.issued2014
dc.identifier.citationZambello R, Teramo A, Gattazzo C, and Semenzato G. Are T-LGL leukemia and NK-chronic lymphoproliferative disorder really two distinct diseases? Translational Medicine @ UniSa 2014;8(1):4-11en_US
dc.identifier.issn2239-9747en_US
dc.identifier.urihttp://hdl.handle.net/10556/1378
dc.identifier.urihttp://dx.doi.org/10.14273/unisa-227
dc.description.abstractMature Large Granular lymphocytes (LGL) disorders include a spectrum of conditions, ranging from polyclonal to clonal indolent and/or overt leukemic LGL proliferations. Most cases are represented by clonal expansions of TCRα/β+ LGL displaying a CD8+ phenotype with expression of cytotoxic T-cell antigens (CD57, CD16, TIA-1, perforin and granzyme B). Proliferations of CD3-CD16+ NK cells with a restricted patter of NK receptors are less common, usually comprising 15% of the cases. Main features are cytopenias, splenomegaly and autoimmune phenomena. Morphology, immunophenotyping and molecular analyses are crucial to establish a correct diagnosis of disease. According to the 2008 WHO classification, two separate entities account for the majority of cases, T-LGL leukemia and Chronic Lymphoproliferative Disease of NK cell (this latter still provisional). Although these disorders are characterized by the expansion of different cells types i.e. T and NK cells, with specific genetic features and abnormalities, compelling evidence supports the hypothesis that a common pathogenic mechanism would be involved in both disorders. As a matter of fact, a foreign antigen driven clonal selection is considered the initial step in the mechanism ultimately leading to generation of both conditions. In this chapter we will discuss recent advances on the pathogenesis of chronic T and NK disorders of granular lymphocytes, challenging the current WHO classification on the opportunity to separate T and NK disorders, which are likely to represent two sides of the same coin.en_US
dc.format.extentP. 4-11en_US
dc.language.isoenen_US
dc.sourceUniSa. Sistema Bibliotecario di Ateneoen_US
dc.subjectCTLen_US
dc.subjectNK cellsen_US
dc.subjectT-LGL leukemiaen_US
dc.subjectCLPD-NKen_US
dc.titleAre T-LGL leukemia and NK-chronic lymphoproliferative disorder really two distinct diseases?en_US
dc.typeArticleen_US
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