Abstract: Objective To compare the differences in clinical characteristics, therapeutic effectiveness and prognosis between extranodal NK/T⁃cell lymphoma, nasal type (ENKTCL) manifesting with primary ocular lesions and ENKTCL with other primary lesions. Methods  The clinical data of 266 newly diagnosed and untreated ENKTCL patients admitted to the Department of Hematology, Beijing Tongren Hospital Affiliated to Capital Medical University from May 2009 to February 2021 were retrospectively analyzed. Combined with the follow⁃up results, the clinical characteristics, therapeutic remission rate and survival between the two groups were compared. Results  Among  the  266  patients  with  ENKTCL, 26 cases manifested with primary ocular lesions, with eyelid swelling being the most common clinical manifestation. Compared with the non⁃ocular ENKTCL groups, the ocular ENKTCL groups had a higher proportion of stage Ⅲ/Ⅳ patients (23.8% vs 42.3%, P=0.0394) and a higher proportion of patients with elevated LDH (27.1% vs 46.2%, P=0.0417). In terms of prognosis, the KPI score≥3 scores of the ocular ENKTCL group was higher than that of the non⁃ocular ENKTCL groups (46.2% vs 21.3%, P=0.0046). The proportion of patients in the ocular ENKTCL group with PINK score at high risk was higher than that in the non⁃ocular ENKTCL group (38.5% vs 19.2%, P=0.0219). In terms of therapeutic effectiveness, there was no significant difference in the choice of treatment regimen between the two groups. The proportion of patients assessed as progressive disease in the ocular ENKTCL group was higher than that in the non⁃ocular ENKTCL groups (46.2% vs 22.9%, P=0.0090). In terms of survival, 12 (46.2%) patients died in the ocular ENKTCL group and 56(23.3%) patients died in the non⁃ocular ENKTCL group. There was no significant difference in the 3⁃year and 5⁃year PFS rates between the two groups; while the 3⁃year OS rates in the ocular and non⁃ocular ENKTCL groups were 69.2% and 76.7%, respectively; the 5⁃year OS rates were 58.6% and 72.2%, respectively, with statistically significant differences (P=0.0321). Conclusions ENKTCL manifesting with primary ocular lesions as the initial clinical manifestation demonstrates distinct features, including high tumor burden, accelerated disease progression, and limited response to therapeutic interventions. The prompt utilization of imaging examinations and tissue biopsy is crucial for precise diagnosis.

Key words: Extranodal NK/T-cell lymphoma, nasal type, Manifesting with primary ocular lesions, Clinical characteristics, Therapeutic effectiveness, Prognosis