Treatments and Outcomes of Patients With Extranodal Natural Killer/T-Cell Lymphoma Diagnosed Between 2000 and 2013: A Cooperative Study in Japan

Yamaguchi, Motoko; Suzuki, Ritsuro; Oguchi, Masahiko; Asano, Naoki; Amaki, Jun; Akiba, Takeshi; Maeda, Takeshi; Itasaka, Satoshi; Kubota, Nobuko; Saito, Yoshihiro; Kobayashi, Yasuhito; Itami, Jun; Ueda, Kyoko; Miyazaki, Kana; Noriko,; Tomita, Naoto; Sekiguchi, Nodoka; Takizawa, Jun; Saito, Bungo; Murayama, Tohru; Ando, Toshihiko; Wada, Hideho; Hyo, Rie; Endo, Yasuo; Hasegawa, Masatoshi; Katayama, Naoyuki

doi:10.1200/jco.2016.68.1619

Cited by 137 publications

(147 citation statements)

References 32 publications

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“…Grade 3/4 febrile neutropenia was observed in 17% of patients and grade 3/4 mucositis in 38%. With a median follow-up of 5.6 years, the 5-year PFS and OS were 61 and 72% [65]. The results were comparable with those observed in previous clinical trials [61, 62].…”

Section: Introductionsupporting

confidence: 81%

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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: Introductionsupporting

confidence: 81%

“…In 150 patients with stage I/II NK/T-cell lymphoma treated with CCRT and DeVIC, the ORR and CR rate was 89 and 82% [65]. Grade 3/4 febrile neutropenia was observed in 17% of patients and grade 3/4 mucositis in 38%.…”

Section: Introductionmentioning

confidence: 99%

The diagnosis and management of NK/T-cell lymphomas

2017

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“…The results of a more recent retrospective analysis (358 patients; 257 patients had localized disease) also reported favorable response and survival rates for patients treated with a concurrent RT-DeVIC regimen. 27 After a median follow-up of 5.6 years, 5-year OS and PFS rates were 72% and 61%, respectively. In this analysis, only 4% of patients with localized disease were classified as high risk according to PINK.…”

Section: Concurrent Chemoradiationmentioning

confidence: 93%

NCCN Guidelines Insights: T-Cell Lymphomas, Version 2.2018

Horwitz

Ansell

et al. 2018

J Natl Compr Canc Netw

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Natural killer (NK)/T-cell lymphomas are a rare and distinct subtype of non-Hodgkin's lymphomas. NK/T-cell lymphomas are predominantly extranodal and most of these are nasal type, often localized to the upper aerodigestive tract. Because extranodal NK/T-cell lymphomas (ENKL) are rare malignancies, randomized trials comparing different regimens have not been conducted to date and standard therapy has not yet been established for these patients. These NCCN Guidelines Insights discuss the recommendations for the diagnosis and management of patients with ENKL as outlined in the NCCN Guidelines for T-Cell Lymphomas.

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“…The 5‐year overall survival (OS) and progression‐free survival (PFS) are 73% and 67%, respectively . On the other hand, the current standard‐of‐care in advanced‐stage ENKL is induction therapy with an intensive L‐asparaginase‐containing regimen, such as dexamethasone, methotrexate, ifosfamide, L‐asparaginase, and etoposide (SMILE) therapy, followed by consolidative therapy with hematopoietic stem cell transplantation; the 5‐year OS and PFS are 47% and 39%, respectively …”

Section: Discussionmentioning

confidence: 99%

EBV‐encoded RNA1‐positive cells in the bone marrow specimens of patients with EBV‐negative lymphomas and sarcomas

Ito¹,

Makita²,

Maeshima³

et al. 2019

Pathology International

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Epstein‐Barr virus (EBV) infection is associated with pathogenesis of various cancers, including extranodal natural killer/T‐cell lymphoma, nasal type (ENKL). ENKL tumor cells are positive for EBV‐encoded RNA1 (EBER1), which is the most useful marker to identify ENKL tumor cells in histopathology. Currently, EBER1 in situ hybridization (ISH) is recommended to evaluate bone marrow (BM) involvement of ENKL. However, the actual burden of EBER1‐positive cells in normal BM specimens remains unclear. In the present study, we performed EBER1 ISH on 111 BM specimens, which were obtained during an initial staging procedure in patients with EBV‐negative cancers and were also negative for BM involvement. One or more EBER1‐positive cells per whole specimen were observed in 38 specimens (34%). The number of EBER1‐positive cells was distributed as follows: single positive cell, n = 17; two positive cells, n = 13; three positive cells, n = 3; and four positive cells, n = 5. These findings suggest that four or fewer EBER1‐positive cells can be observed in BM specimens of patients with non‐EBV‐related cancers. The clinical implications of a small number of EBER1‐positive cells in BM specimens of patients with ENKL should be evaluated in further studies.

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Treatments and Outcomes of Patients With Extranodal Natural Killer/T-Cell Lymphoma Diagnosed Between 2000 and 2013: A Cooperative Study in Japan

Cited by 137 publications

References 32 publications

The diagnosis and management of NK/T-cell lymphomas

The diagnosis and management of NK/T-cell lymphomas

NCCN Guidelines Insights: T-Cell Lymphomas, Version 2.2018

EBV‐encoded RNA1‐positive cells in the bone marrow specimens of patients with EBV‐negative lymphomas and sarcomas

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