Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis

• Type: Another Publication in an International Scientific Journal
• Date: 2013

Authors

Lima, M (Author) ICBAS

Journal

Title: Orphanet Journal of Rare Diseases

Vol. 8

Final page: 95

ISSN: 1750-1172

Publisher: Springer Nature

Indexing

ISI Web of Knowledge - 36 Citations

Scopus - 14 Citations

Other information

Authenticus ID: P-006-9QW

DOI: 10.1186/1750-1172-8-95

Abstract (EN): 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.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 10