Blood Res 2020; 55(S1):
Published online July 31, 2020
https://doi.org/10.5045/br.2020.S011
© The Korean Society of Hematology
Correspondence to : Seong Hyun Jeong, M.D.
Department of Hematology-Oncology, Ajou University School of Medicine, 21, World cup-ro 150beon-gil, Yeongtong-gu, Suwon 16500, Korea
E-mail: seonghyunmd@naver.com
This is an Open Access article distributed unAcute myeloid leukemia, New FDA approvalsder the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Extranodal natural killer (NK)/T cell lymphoma (ENKTL) is a distinct subtype of Non-Hodgkin’s lymphoma mainly involving the nasal area. Since the entity was first recognized, treatment strategies have been evolving from anthracycline-based chemotherapy and radiotherapy to L-asparaginase containing regimens and recently immune checkpoint inhibitors. With the currently used combined chemotherapy and radiotherapy, more than 70% of patients with localized disease can be cured. L-asparaginase containing regimens have significantly improved treatment outcomes among patients with advanced disease. However, the treatment outcomes of patients with disease refractory to L-asparaginase containing regimens or who experience recurrence remain poor. In this article, we cover the current treatments for ENKTL and emerging treatment approaches.
Keywords Extranodal natural killer/T cell lymphoma, ENKTL, Non-Hodgkin’s lymphoma, Immunotherapy
Blood Res 2020; 55(S1): S63-S71
Published online July 31, 2020 https://doi.org/10.5045/br.2020.S011
Copyright © The Korean Society of Hematology.
Seong Hyun Jeong
Department of Hematology-Oncology, Ajou University School of Medicine, Suwon, Korea
Correspondence to:Seong Hyun Jeong, M.D.
Department of Hematology-Oncology, Ajou University School of Medicine, 21, World cup-ro 150beon-gil, Yeongtong-gu, Suwon 16500, Korea
E-mail: seonghyunmd@naver.com
This is an Open Access article distributed unAcute myeloid leukemia, New FDA approvalsder the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Extranodal natural killer (NK)/T cell lymphoma (ENKTL) is a distinct subtype of Non-Hodgkin’s lymphoma mainly involving the nasal area. Since the entity was first recognized, treatment strategies have been evolving from anthracycline-based chemotherapy and radiotherapy to L-asparaginase containing regimens and recently immune checkpoint inhibitors. With the currently used combined chemotherapy and radiotherapy, more than 70% of patients with localized disease can be cured. L-asparaginase containing regimens have significantly improved treatment outcomes among patients with advanced disease. However, the treatment outcomes of patients with disease refractory to L-asparaginase containing regimens or who experience recurrence remain poor. In this article, we cover the current treatments for ENKTL and emerging treatment approaches.
Keywords: Extranodal natural killer/T cell lymphoma, ENKTL, Non-Hodgkin’s lymphoma, Immunotherapy
Table 1 . Treatment strategies for localized (stage IE/IIE) ENKTL..
Regimen | Treatment | N | ORR/CR (%) | OS/PFS (%) | Grade III/IV neutropenia (%) |
---|---|---|---|---|---|
Concurrent therapy | |||||
DeVIC [38, 39] | Simultaneous RT 50 Gy+DeVIC×3 | 33 | 77/76 | 70/63 (5-yr) | 91 |
VIPD [40] | CCRT (weekly cisplatin+RT 40–50.8 Gy) | 30 | 83/80 | 86/85 (3-yr) | 47 |
→VIPD×3 | |||||
VIDL [41] | CCRT (weekly cisplatin+RT 40–44Gy) | 30 | 90/87 | 60/73 (5-yr) | 80 |
→VIDL×3 | |||||
MIDDLE [42] | CCRT (weekly cisplatin, L-asparaginase×3+RT 36–44 Gy)→MIDDLE×2 | 28 | 86/82 | 82/74 (3-yr) | 91 |
GDP [43] | CCRT (weekly cisplatin+IMRT 56 Gy)→GDP×3 | 32 | 91/84 | 88/84 (3-yr) | 41 (leukopenia) |
MPVIC-P [44] | Simultaneous RT 56 Gy+intra-arterial | 12 | 100/100 | 100/100 (5-yr) | 33 |
MPVIC-P | |||||
Sequential therapy | |||||
SMILE [44] | SMILE→RT→SMLIE | 17 | NA/82 | NA | NA |
LVP [32, 47] | LVP→IFRT→LVP | 26 | 89/81 | 65/64 (5-yr) | 31 (grade 3) |
GELOX [31, 48] | GELOX→IFRT | 27 | 96/74 | 86/86 (2-yr) | NA |
P-GEMOX [49] | P-GEMOX×2→RT 56 Gy | 33 | 94/80 | 83/77 (2-yr) | 33.3 |
DICE-L [50] | DICE-L×3→RT 45 Gy | 27 | NA/91 | 89/82 (5-yr) | 4.4 (leukopenia) |
Abbreviations: CR, complete remission; ENKTL, Extranodal NK/T cell lymphoma; NA, not available; ORR, overall response rate; OS, overall survival; PFS, progression-free survival; PR, partial remission; RT, radiotherapy..
Table 2 . New treatment targets for ENKTL..
Target | Treatment | Patients | Outcome | Reference |
---|---|---|---|---|
PD-1/PD-L1 | Pembrolizumab | 7 relapsed | 5 CR, 2 PR | [69] |
Nivolumab | 3 relapsed | 3 CR | [71] | |
CD38 | Daratumumab | 2 RR | 1 CR, 1 PR | [74, 75] |
CD30 | BV | 2 refractory | 2 CR | [79, 80] |
CCR4 | Mogamulizumab | Preclinical | NA | [85] |
LMP1/LMP2 | Autologous CTLs | 11 ENKTL6 active disease5 in CR | 9 CR 4 CR 5 remained CR | [86] |
Autologous CTLs | 10 ENKTL in CR | 9 remained CR | [87] | |
JAK/STAT | Vorinostat | 1 pediatric ENKTL | CR | [89] |
Ruxolitinib | Clinical trial | Ongoing | [NCT02974647] |
Abbreviations: BV, brentuximab vedotin; CR, complete remission; CTL, cytotoxic T lymphocyte; ENKTL, extranodal NK/T cell lymphoma; PR, partial remission; RR, refractory/relapsed..
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