Intravascular NK/T-Cell Lymphoma: What We Know about This Diagnostically Challenging, Aggressive Disease.
EBV-positive primary nodal T/NK-cell lymphoma
Epstein–Barr virus
aggressive NK-cell leukemia
extranodal NK/T-cell lymphoma
intravascular NK/T-cell lymphoma
Journal
Cancers
ISSN: 2072-6694
Titre abrégé: Cancers (Basel)
Pays: Switzerland
ID NLM: 101526829
Informations de publication
Date de publication:
06 Nov 2022
06 Nov 2022
Historique:
received:
24
10
2022
revised:
02
11
2022
accepted:
05
11
2022
entrez:
11
11
2022
pubmed:
12
11
2022
medline:
12
11
2022
Statut:
epublish
Résumé
Intravascular lymphoma is a form of lymphoid malignancy characterized by neoplastic cells growing almost exclusively within the lumina of small- to medium-sized blood vessels. Most cases are of B-cell origin with rare cases of natural killer or T-cell lineage. Extranodal sites are affected, mainly the skin and central nervous system, although any organ may be involved. Intravascular NK/T-cell lymphoma deserves special attention because of its clinicopathologic features and the need for adequate immunophenotyping combined with clonality test for a proper diagnosis. Moreover, intravascular NK/T-cell lymphoma is strongly linked to Epstein-Barr virus (EBV), which is considered to play a role in tumorigenesis and to be responsible for the aggressive behavior of the disease. In this paper, we review the current knowledge on this rare lymphoma and, in particular, the most recent advances about its molecular landscape. The main distinguishing features with other EBV-related entities, such as extranodal NK/T-cell lymphoma, EBV-positive primary nodal T/NK-cell lymphoma, and aggressive NK-cell leukemia, are discussed to help pathologists obtain the correct diagnosis and consequently develop an adequate and prompt therapy response.
Identifiants
pubmed: 36358876
pii: cancers14215458
doi: 10.3390/cancers14215458
pmc: PMC9658079
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
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