Diagnosis and management of extranodal NK/T cell lymphoma nasal type

Tse, Eric; Kwong, Yok–Lam

doi:10.1080/17474086.2016.1206465

Cited by 72 publications

(78 citation statements)

References 84 publications

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“…The neoplastic infiltrate comprised a polymorphic population of atypical lymphoid cells, inflammatory cells, and eosinophils, which was quite different from conventional lymphomas where neoplastic cells were more homogeneous. Hence, the notation of polymorphic reticulosis was adopted [6]. The development of immunohistochemistry further improved the classification of this lymphoma.…”

Section: Introductionmentioning

confidence: 99%

“…The CD3 detected previously with polyclonal antibodies on paraffin-embedded sections represents detection of the cytoplasmic ε chain of CD3 [8], a characteristic of NK-cells instead. Finally, molecular analysis of the T-cell receptor (TCR) gene shows a germline configuration in the majority of cases [6]. Therefore, these lymphomas are in fact of putative NK-cell origin.…”

Section: Introductionmentioning

confidence: 99%

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The diagnosis and management of NK/T-cell lymphomas

2017

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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.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The diagnosis and management of NK/T-cell lymphomas

2017

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“…1,4 However, SMILE or SMILE-like regimens still fail in 20% to 40% of cases. [1][2][3] There is no known effective salvage, and outcome is virtually always fatal.…”

Section: Introductionmentioning

confidence: 99%

PD1 blockade with pembrolizumab is highly effective in relapsed or refractory NK/T-cell lymphoma failing l-asparaginase

Kwong

Chan

Tan

et al. 2017

Blood

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“…However, as we mentioned previously, ENKTL is an uncommon disease and its chemotherapy regimens still remain controversial. For the last decades, no effective novel drug has been available [36], and the anthracycline-based chemotherapy regimens that have high similarity are recommended for most ENKTL patients [37,38]. Furthermore, the addition of chemotherapy to RT does not change the survival outcomes of patients with early-stage ENKTL [24,25,[39][40][41][42][43][44].…”

Section: Discussionmentioning

confidence: 99%