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Current Hematologic Malignancy Reports

, Volume 13, Issue 1, pp 7–12 | Cite as

NK/T Cell Lymphoma: Updates in Therapy

  • Ritsuro Suzuki
T-Cell and Other Lymphoproliferative Malignancies (J Zain, Section Editor)
Part of the following topical collections:
  1. Topical Collection on T-Cell and Other Lymphoproliferative Malignancies

Abstract

Purpose of Review

Extranodal NK/T cell lymphoma (ENKL), nasal type, is a highly aggressive lymphoma which used to show a poor clinical outcome. Expression of P-glycoprotein on lymphoma cells of ENKL is a major reason for the refractoriness to conventional chemotherapy containing anthracycline. However, recent innovative approaches have improved the outcome and prognosis of ENKL. The purpose of this review is to summarize the proceedings of treatment.

Recent Findings

Concurrent chemoradiotherapy containing platinum and several drugs including L-asparaginase, methotrexate, and alkylators shows excellent outcomes for the limited-stage ENKL. SMILE (steroid, methotrexate, ifosfamide, L-asparaginase, and etoposide) or other L-asparaginase-containing therapy is promising for advanced-stage ENKL, followed by either autologous or allogeneic hematopoietic stem cell transplantation. Anti-PD-1 or other immunological checkpoint inhibitors are recently reported to be effective for relapsed/refractory ENKL thought to be due to EBV-driven upregulation of PD-L1 expression.

Summary

The prognosis of ENKL is therefore improving by the introduction of these strategies. The 5-year overall survival (OS) rate of limited stage was 63.2% [95% confidence interval (CI), 55.3 to 70.0%] before 2010, but was 79.4% (95% CI, 66.9 to 87.6%) in 2010 or after. However, there still exists a room for improvement, particularly for advanced-stage patients. The 2-year OS of advanced ENKL was 30.3% (95% CI, 19.5 to 41.7%) before 2010, but was 40.5% (95% CI, 24.8 to 55.8%) in 2010 or after. Optimal treatment scheme should further be explored.

Keywords

Extranodal NK cell lymphoma Concurrent radiotherapy Chemotherapy L-asparaginase 

Notes

Compliance with Ethical Standards

Conflict of Interest

Ritsuro Suzuki reports personal fees from Chugai Pharmaceutical, personal fees from Kyowa-Hakko Kirin Pharmaceutical, personal fees from Takeda Pharmaceutical, personal fees from Meiji Seika Pharma, personal fees from Bristol-Meyers Squib, personal fees from Celgene Inc., personal fees from MSD Inc., personal fees from Sanofi Inc., personal fees from Gilead Sciences, personal fees from Mundi Pharma, personal fees from Jazz Pharma, personal fees from Mochida Pharma, personal fees from Novartis Pharma, personal fees from Sysmex Inc., and personal fees from Sumitomo Dainippon.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Authors and Affiliations

  1. 1.Department of Oncology and HematologyShimane University Hospital Cancer CenterIzumoJapan

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