以眼部肿物为首发表现的鼻型结外NK/T细胞淋巴瘤的临床分析

Clinical analysis of extranodal NK/T⁃cell lymphoma,nasal type,manifesting with primary ocular lesions

目的 比较以眼部肿物为首发表现的鼻型结外NK/T细胞淋巴瘤(extranodal NK/T-cell lymphoma,nasal type,ENKTCL),与其他部位首发的ENKTCL临床特征、疗效及预后的差异。方法 回顾性分析2009年5月至2021年2月于首都医科大学附属北京同仁医院血液科收治的266例新诊断且未经治疗的ENKTCL患者的临床资料,结合随访调查,对两组患者的临床特征、治疗后缓解率及生存情况进行比较分析。结果 266例ENKTCL患者中,26例患者以眼部肿物为首发表现,其中眼睑肿胀为最常见的临床表现。与其他ENKTCL组相比,眼部ENKTCL组中Ⅲ/Ⅳ期患者所占比例更高(23.8%vs 42.3%,P=0.0394),LDH升高患者的比例更高(27.1%vs 46.2%,P=0.0417)。预后方面,眼部ENKTCL组KPI评分≥3分的患者比例高于其他ENKTCL组(46.2%vs 21.3%,P=0.0046);眼部ENKTCL组PINK评分为高危组的患者比例高于其他ENKTCL组(38.5%vs 19.2%,P=0.0219)。疗效方面,两组患者在治疗方案选择上差异无统计学意义;眼部ENKTCL组评效为疾病进展的患者比例高于其他ENKTCL患者组(46.2%vs 22.9%,P=0.0090)。生存方面,眼部ENKTCL组中死亡患者共有12(46.2%)例,其他ENKTCL组中有56(23.3%)例。两组患者3年及5年的PFS率差异无统计学意义,但眼部和其他ENKTCL组3年的OS率分别为69.2%和76.7%,以及5年的OS率分别为58.6%和72.2%,差异有统计学意义(P=0.0321)。结论 以眼部肿物为首发临床表现的ENKTCL具有高肿瘤负荷、疾病进展快、对治疗的反应不佳及预后差等特点,及时进行影像学检查及组织活检对明确诊断尤为重要。

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 stageⅢ/Ⅳpatients(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 athigh risk was higher than thatin 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.