结外鼻型NK/T细胞淋巴瘤中枢神经系统侵犯的临床特征及治疗结果

Clinical characteristics and treatment outcome analysis of central nervous system involvement in extranodal natural killer/T-cell lymphoma

目的分析结外鼻型NK/T细胞淋巴瘤(NKTCL)中枢神经系统侵犯病例的临床特征、治疗策略及结果。方法从2010——2021年于我院治疗的1019例NKTCL患者中纳入中枢侵犯患者14例进行分析。结果NKTCL中枢侵犯发病率1.37%。5例初诊时出现中枢侵犯,9例进展时出现中枢侵犯。治疗后完全缓解11例,部分缓解1例。患者达完全缓解时接受大剂量甲氨蝶呤方案5例、培门冬酶方案4例、PD-1单抗方案3例、西达苯胺方案2例。诊断中枢侵犯后的中位随访时间为24.5个月,中位总生存时间未达到。结论本研究中患者的治疗效果及生存预后较既往研究有所提升,提示甲氨蝶呤及培门冬酶等化疗药物常规应用于NKTCL中枢侵犯的价值,而抗PD-1单抗及西达本胺等新型药物的应用可能进一步提升了患者预后。

Objective The clinical characteristics,treatment strategy and results of the central nervous system(CNS)invasion cases of extranodal nasal-type NK/T-cell lymphoma(NKTCL)were analyzed.Methods 1019 patients diagnosed with extranodal NKTCL,from 2010 to 2021 were reviewed,and 14 patients with CNS involvement were included in the analysis.Results The incidence of NKTCL central invasion was 1.37%.The 5 patients suffered CNS involvement at initial diagnosis of NKTCL.The 9 patients involvement were identified as disease progression.After systemic treatment,there were 11 cases of complete response and 1 case of partial response.The complete response was achieved when patients were treated with HD-MTX-containing regimen(n=5),Pegaspargase-containing regimen(n=4),anti-PD-1 antibodies-containing regimen(n=3)and Chidamide-containing regimen(n=2).After the median follow-up of 24.5 months,the median overall survival was not reached.Conclusions Compared with previous studies,the treatment effect and survival outcome of patients in this study were improved.These results suggest that chemotherapeutic drugs,such as methotrexate and Pegaspargase played a role in the treatment for NKTCL patients with CNS involvement,further more,anti-PD-1 antibodies and Chidamide may contribute to the improved clinical outcomes further.