Publication: Natural killer cell malignancies: clinicopathologic and molecular features
Authors
Siu, L.L.P. ; Chan, John K.C. ; Kwong, Y.L.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Malignancies of natural killer (NK) cells
h ave increasingly been recognized as distinct
clinicopathological entities. The tumor cells are
characterized by an immunophenotype of CD2+, surface
CD3-, cytoplasmic CD3e+, and CD56+. The T cell
receptor gene is in germline configuration, and a
consistent association with Epstein-Barr virus is
demonstrable. Pa t h o l o g i c a l l y, the tumor cells show
variable cytological appearances, with frequent
angioinvasion and angiocentricity associated with zonal
necrosis. Clinically, most cases affect the nasal cavity or
other parts of the upper aerodigestive tract, and are
referred to as nasal NK cell lymphoma. A minority
involve extranasal sites such as the skin, gastrointestinal
tract and testis, and are often referred to as ex t r a n a s a l
NK cell lymphoma. A particularly aggressive form
presents fulminantly as disseminated disease, sometimes
with a leukemic phase, and is referred to as aggressive
NK cell lymphoma/leukemia. Cytogenetic and molecular
analysis have shown DNA losses at chromosomes 6q,
11q, 13q and 17p to be recurrent aberrations in NK cell
malignancies. Frequent DNA gains are also found in
chromosomes 1p, 6p, 11q, 12q, 17q, 19p, 20q, and Xp.
These regions of DNA losses and gains should be targets
for further inve s t i gation in order to understand the
molecular pathogenesis of this lymphoma. Finally,
optimal treatment modalities need to be determined, as
all subtypes of NK cell malignancies are associated with
a poor prognosis.
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