Publication
Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis
dc.contributor.author | Lima, M. | |
dc.date.accessioned | 2014-10-14T13:35:14Z | |
dc.date.available | 2014-10-14T13:35:14Z | |
dc.date.issued | 2013 | |
dc.description.abstract | Mature 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.citation | Lima Orphanet Journal of Rare Diseases 2013, 8:95 | por |
dc.identifier.doi | 10.1186/1750-1172-8-95 | |
dc.identifier.issn | 1750-1172 | |
dc.identifier.uri | http://hdl.handle.net/10400.16/1692 | |
dc.language.iso | eng | por |
dc.peerreviewed | yes | por |
dc.publisher | BioMed Central | por |
dc.relation.publisherversion | http://www.ojrd.com/content/8/1/95 | por |
dc.subject | NK-cell Neoplasms | por |
dc.subject | NK/T-cell Lymphoma | por |
dc.subject | Nasal-type | por |
dc.subject | Aggressive NK-Cell Leukemia | por |
dc.subject | CD56 | por |
dc.title | Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis | por |
dc.type | journal article | |
dspace.entity.type | Publication | |
oaire.citation.conferencePlace | England | por |
oaire.citation.title | Orphanet journal of rare diseases | por |
oaire.citation.volume | 8 | por |
rcaap.rights | openAccess | por |
rcaap.type | article | por |
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