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International Journal of Hematology
/10.1007/s12185-019-02609-x
GUIDE LINE
JSH practical guidelines for hematological malignancies, 2018: II.
Lymphoma-9. Extranodal NK/T-cell lymphoma, nasal type (ENKL)
1 2
Motoko Yamaguchi · Ritsuro Suzuki
Received: 24 January 2019 / Accepted: 24 January 2019
© Japanese Society of Hematology 2019
Overview been proposed in Japan and South Korea [2–4]. Unlike with
other lymphomas, age at diagnosis is not a prognostic fac-
The 2017 WHO Classifcation lists three entities under natu- tor. Clinical staging is critical to treatment selection. For
ral killer (NK) cell neoplasms: extranodal NK/T-cell lym- response assessment, 18fluoro-2-deoxyglucose positron
phoma, nasal type (ENKL); aggressive NK-cell leukemia emission tomography (FDG-PET) is useful because the
(ANKL); and chronic lymphoproliferative disorders of NK nasal area is particularly anatomically complex, non-neo-
cells (CLPD-NK) [1]. All entities are rare, but the incidence plastic tissue such as hypertrophic mucosa can remain even
of ANKL is less than one-seventh that of ENKL [2], there- when the tumor is eliminated, and ENKL has a high rate of
fore, there is little evidence regarding its treatment. Conse- FDG uptake on PET [5].
quently, this chapter only covers ENKL.
ENKL is almost always derived from NK cells, but a
small number of cases that originate in or around the nasal References
cavity (hereafter collectively referred to as nasal ENKL)
are considered to be lymphomas derived from T cells. As it 1. Chan JKC, et al. Extranodal NK/T-cell lymphoma, nasal
is not possible to diferentiate between the NK- and T-cell type. Sw
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