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中国癌症防治杂志

中国癌症防治杂志 ›› 2025, Vol. 17 ›› Issue (1): 81-87.doi: 10.3969/j.issn.1674-5671.2025.01.11

• 临床研究 • 上一篇    下一篇

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效果分析

  

  1. 广西壮族自治区人民医院肿瘤内科一区；广西壮族自治区人民医院老年内分泌代谢科；广西壮族自治区人民医院广西医学科学院科研合作部
  • 出版日期:2025-02-25 发布日期:2025-03-06
  • 通讯作者: 杨磊 E-mail：echosun1101@163.com
  • 基金资助:
    国家自然科学基金项目（82170181；82370188）

Clinical features and treatment efficacy analysis of central nervous system involvement in extranodal nasal-type natural killer/T-cell lymphoma

  • Online:2025-02-25 Published:2025-03-06

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摘要： 目的 分析结外鼻型NK/T细胞淋巴瘤（extranodal nasal⁃type NK/T cell lymphoma，ENKTCL）中枢神经系统（central nervous system，CNS）侵犯病例的临床特征、诊疗策略及生存结局。方法 回顾性分析2009年5月至2023年11月于首都医科大学附属北京同仁医院收治的18例发生CNS侵犯的ENKTCL患者的临床及影像学资料。结合随访调查，对患者的临床特征、治疗后缓解率及预后情况进行分析。结果 18例出现CNS侵犯的ENKTCL患者的中位年龄为48岁（33~61岁），其中72.2%（13/18）为男性。从诊断ENKTCL到出现CNS侵犯的中位时间为4个月（1~24个月），6例患者在初诊时已伴有CNS侵犯，其余12例在疾病进展时出现。影像学检查结果显示， 15例患者可见明显CNS病灶；16例患者接受脑脊液检查，其中13例出现脑脊液异常；另有2例患者房水检测可见淋巴瘤细胞，且光学相干断层扫描检查可见淋巴瘤受累典型表现。治疗结局方面，16例患者接受了以大剂量甲氨蝶呤和培门冬酶为基础的化疗，2例达到完全缓解，1例部分缓解，15例病情进展。诊断CNS侵犯后的中位随访时间为11.4个月（1~25个月），中位总生存时间为3.5个月。结论 ENKTCL CNS侵犯非常罕见，具有疾病进展快、预后差等特点。结合临床表现、影像学、脑脊液及房水检查进行综合诊断，有助于及时发现CNS病灶，从而实施有效的个体化治疗方案以改善患者预后。

关键词:  , NK/T细胞淋巴瘤；中枢神经系统侵犯；临床特征；治疗效果

Abstract: Objective To analyze the clinical features, treatment strategies and survival outcomes of extranodal nasal⁃type NK/T⁃cell lymphoma (ENKTCL) cases involving central nervous system (CNS) involvement. Methods The clinical and imaging data of 18 ENKTCL patients with CNS involvement admitted to Beijing Tongren Hospital, Capital Medical University from May 2009 to November 2023 were retrospectively analyzed. Combined with follow⁃up investigation, the clinical features, remission rate and prognosis of patients after treatment were analyzed. Results Among the 18 ENKTCL patients with CNS involvement, the median age was 48 years (range, 33-61 years), with 72.2% (13/5) being male. The median time from initial diagnosis of ENKTCL to CNS involvement was 4 months(range, 1-24 months), 6 patients suffered CNS involvement at the time of initial ENKTCL diagnosis, while the remaining patients  were identified during disease progression. Radiological examinations identified CNS lesion locations in 15 patients. 13 of 16 patients displayed abnormal cerebrospinal fluid (CSF) underwent CSF examinations. Lymphoma cells were detected in the aqueous humor of 2 patients, and lymphoma involvement was identified using optical coherence tomography. In terms of treatment outcomes, 16 patients received high⁃dose methotrexate and pegaspargase⁃based chemotherapy, 2 achieved a complete response, 1 had a partial response and 15 experienced progressive disease. After the median follow⁃up of 11.4 months (range, 1⁃25 months), the median overall survival was 3.5 months. Conclusions CNS involvement in ENKTCL is a rare condition characterized by rapid disease progression and poor prognosis. The comprehensive diagnosis combined with clinical manifestations, the results of radiological and CSF examinations and aqueous humor, it aids in identifying the central lesion, developing an individualized treatment plan, and improving patient prognosis.

Key words:  Extranodal nasal?type NK/T?cell lymphoma, Central nervous system involvement, Clinical features, Treatment efficacy 

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