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Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis

2013Journal article Open access
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dc.contributor.authorLima, M.
dc.date.accessioned2014-10-14T13:35:14Z
dc.date.available2014-10-14T13:35:14Z
dc.date.issued2013
dc.description.abstractMature natural killer (NK) cell neoplasms are classified by the World Health Organization into NK/T cell lymphoma, nasal type (NKTCL), aggressive NK-cell leukemia (ANKCL) and chronic lymphoproliferative disorders of NK-cells, the latter being considered provisionally. NKTCL and ANKCL are rare diseases, with higher prevalence in Asia, Central and South America. Most NKTCL present extranodal, as a destructive tumor affecting the nose and upper aerodigestive tract (nasal NKTCL) or any organ or tissue (extranasal NKTCL) whereas ANKCL manifests as a systemic disease with multiorgan involvement and naturally evolutes to death in a few weeks. The histopathological hallmark of these aggressive NK-cell tumors is a polymorphic neoplastic infiltrate with angiocentricity, angiodestruction and tissue necrosis. The tumor cells have cytoplasmatic azurophilic granules and usually show a CD45+bright, CD2+, sCD3-, cytCD3epsilon+, CD56+bright, CD16−/+, cytotoxic granules molecules+ phenotype. T-cell receptor genes are in germ-line configuration. Epstein-Barr virus (EBV) -encoded membrane proteins and early region EBV RNA are usually detected on lymphoma cells, with a pattern suggestive of a latent viral infection type II. Complex chromosomal abnormalities are frequent and loss of chromosomes 6q, 11q, 13q, and 17p are recurrent aberrations. The rarity of the NK-cell tumors limits our ability to standardize the procedures for the diagnosis and clinical management and efforts should be made to encourage multi-institutional registries.por
dc.identifier.citationLima Orphanet Journal of Rare Diseases 2013, 8:95por
dc.identifier.doi10.1186/1750-1172-8-95
dc.identifier.issn1750-1172
dc.identifier.urihttp://hdl.handle.net/10400.16/1692
dc.language.isoengpor
dc.peerreviewedyespor
dc.publisherBioMed Centralpor
dc.relation.publisherversionhttp://www.ojrd.com/content/8/1/95por
dc.subjectNK-cell Neoplasmspor
dc.subjectNK/T-cell Lymphomapor
dc.subjectNasal-typepor
dc.subjectAggressive NK-Cell Leukemiapor
dc.subjectCD56por
dc.titleAggressive mature natural killer cell neoplasms: from epidemiology to diagnosispor
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceEnglandpor
oaire.citation.titleOrphanet journal of rare diseasespor
oaire.citation.volume8por
rcaap.rightsopenAccesspor
rcaap.typearticlepor

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