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19.04.2019 | Original Article | Ausgabe 7/2019

Annals of Hematology 7/2019

Improved prognosis of extranodal NK/T cell lymphoma, nasal type of nasal origin but not extranasal origin

Zeitschrift:
Annals of Hematology > Ausgabe 7/2019
Autoren:
Motoko Yamaguchi, Ritsuro Suzuki, Kana Miyazaki, Jun Amaki, Jun Takizawa, Nodoka Sekiguchi, Shiori Kinoshita, Naoto Tomita, Hideho Wada, Yukio Kobayashi, Nozomi Niitsu, Toshihiko Ando, Takeshi Maeda, Bungo Saito, Hiroshi Matsuoka, Rika Sakai, Nobuko Kubota, Yasufumi Masaki, Yoshihiro Kameoka, Naoko Asano, Masahiko Oguchi, Naoyuki Katayama
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The online version of this article (https://​doi.​org/​10.​1007/​s00277-019-03689-9) contains supplementary material, which is available to authorized users.

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Abstract

Extranodal NK/T cell lymphoma (NKTCL), nasal type (ENKL) that shows no apparent nasal involvement, is termed extranasal NKTCL or non-nasal NKTCL. In this study, we aimed to explore therapeutic approaches and outcomes in patients with extranasal NKTCL in current clinical practice. A data set of patients with newly diagnosed NKTCL who were diagnosed at 31 institutes in Japan between 2000 and 2013 was used for analysis. The patients’ fitness for steroid, methotrexate, ifosfamide, l-asparaginase, and etoposide (SMILE) chemotherapy was assessed using the major inclusion criteria of the SMILE phase 2 study. Of 358 patients, 47 (13%) had extranasal NKTCL. The most frequent extranodal sites of involvement in extranasal NKTCL were skin/subcutaneous tissue (n = 18). Six (13%) of the patients with extranasal NKTCL had localized disease and were diagnosed before 2010. With a median follow-up of 5.8 years, the 2-year overall survival (OS) in patients with nasal and extranasal NKTCL was 70% (95% confidence interval [CI], 65–75%) and 34% (95% CI, 21–47%), respectively. OS in patients with nasal NKTCL had a trend toward better according to treatment era (P = 0.063). In contrast, no obvious improvement of OS was observed in extranasal NKTCL (P = 0.43). The major inclusion criteria of the SMILE-P2 were met in 21% (10/47) of patients with extranasal NKTCL and 60% (188/311) of those with nasal NKTCL (P < 0.001). Despite the advent of new treatments for ENKL, OS remains unfavorable in extranasal NKTCL. A more effective therapy is needed for extranasal NKTCL.

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