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2017 ; 10
(1
): 85
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The diagnosis and management of NK/T-cell lymphomas
#MMPMID28410601
Tse E
; Kwong YL
J Hematol Oncol
2017[Apr]; 10
(1
): 85
PMID28410601
show ga
Extranodal natural killer (NK)/T-cell lymphoma is an aggressive malignancy of
putative NK-cell origin, with a minority deriving from the T-cell lineage.
Pathologically, the malignancy occurs in two forms, extranodal NK/T-cell
lymphoma, nasal type; and aggressive NK-cell leukaemia. Lymphoma occur most
commonly (80%) in the nose and upper aerodigestive tract, less commonly (20%) in
non-nasal areas (skin, gastrointestinal tract, testis, salivary gland), and
rarely as disseminated disease with a leukemic phase. Genetic analysis showed
mutations of genes involved in the JAK/STAT pathway, RNA assembly, epigenetic
regulation, and tumor suppression. In initial clinical evaluation, positron
emission tomography computed tomography, and quantification of plasma EBV DNA are
mandatory as they are useful for response monitoring and prognostication. In
stage I/II diseases, combined chemotherapy and radiotherapy (sequentially or
concurrently) is the best approach. Conventional anthracycline-containing
regimens are ineffective and should be replaced by non-anthracycline-containing
regimens, preferably including L-asparaginase. Radiotherapy alone is associated
with high systemic relapse rates and should be avoided. In stage III/IV diseases,
non-anthracycline-regimens-containing L-asparaginase are the standard. In
relapsed/refractory cases, blockade of the programmed death protein 1 has
recently shown promising results with high response rates. In the era of
effective non-anthracycline-containing regimens, autologous haematopoietic stem
cell transplantation (HSCT) has not been shown to be beneficial. However,
allogeneic HSCT may be considered for high-risk or advanced-stage patients in
remission or relapsed/refractory patients responding to salvage therapy.
Prognostic models taking into account presentation, interim, and end-of-treatment
parameters are useful in triaging patients to different treatment strategies.