2018JSH实践指南_血液恶性肿瘤-成人T细胞白血病-淋巴瘤-9结节外NK／T细胞淋巴瘤(鼻型).pdf

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 Classiication 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