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Fujimoto et al. J Cancer Metastasis Treat 2021;7:66 Journal of Cancer
DOI: 10.20517/2394-4722.2021.157
Metastasis and Treatment

Review Open Access

Progress in the treatment of NK-cell
lymphoma/leukemia

Ayumi Fujimoto, Ritsuro Suzuki

Department of Hematology, Shimane University Hospital, Izumo, Shimane 693-8501, Japan.
Correspondence to: Dr. Ritsuro Suzuki, Department of Hematology, Shimane University Hospital, 89-1 En-ya cho, Izumo,
Shimane 693-8501, Japan. E-mail: rsuzuki@med.shimane-u.ac.jp

How to cite this article: Fujimoto A, Suzuki R. Progress in the treatment of NK-cell lymphoma/leukemia. J Cancer Metastasis Treat
2021;7:66. https://dx.doi.org/10.20517/2394-4722.2021.157

Received: 4 Aug 2021 First Decision: 14 Sep 2021 Revised: 29 Sep 2021 Accepted: 29 Oct 2021 Published: 5 Dec 2021

Academic Editors: Lucio Miele, Lorenzo Leoncini, Ivory Ma Copy Editor: Xi-Jun Chen Production Editor: Xi-Jun Chen

Abstract
Natural killer (NK)/T cell lymphoma includes two major subtypes of disease, specifically extranodal NK/T cell
lymphoma, nasal type (ENKL) and aggressive NK cell leukemia (ANKL). Both are strongly associated with Epstein-
Barr virus and are prevalent in East Asia and Latin America. Except for that of limited-stage ENKL, the prognosis of
both diseases was poor in the previous decade. The advent of non-anthracycline-based chemoradiotherapy has
contributed to an improvement in ENKL prognosis, but there is still room for further treatment progress. Recently,
the high efficacy of PD-1 antibody was reported in relapsed or refractory ENKL patients. This was later supported
by the finding that PD-L1/PD-L2 genetic alterations are frequently observed in ENKL and ANKL patients. Due to the
rarity of the disease, a standard treatment for ANKL remains to be established. Currently, allogeneic stem cell
transplantation is the only curative treatment, and this is even applicable to chemo-resistant ANKL patients. In this
review, we focus on recent treatment approaches for NK/T cell lymphomas including novel agents.

Keywords: Extranodal NK/T-cell lymphoma, nasal type, aggressive NK-cell leukemia, Epstein-Barr virus,
hematopoietic stem cell transplantation, L-asparaginase

INTRODUCTION
Natural killer (NK) cell neoplasms are rare hematological malignancies, with two major subtypes of
extranodal NK/T cell lymphoma, nasal type (ENKL) and aggressive NK-cell leukemia (ANKL) . Both
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© The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0
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