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病例报告:伴广泛中枢神经系统受累的复发/难治性鼻型结外自然杀伤/T 细胞淋巴瘤

英文原题:Case report: Relapsed/refractory extranodal natural killer/T-cell lymphoma nasal type with extensive central nervous system involvement.

查看英文原题

Case report: Relapsed/refractory extranodal natural killer/T-cell lymphoma nasal type with extensive central nervous system involvement.

PubMed 2023/01/09(内容时间) Pathol Oncol Res Q2 · IF 2.7(JCR 2025)

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中文摘要

结外自然杀伤/T细胞淋巴瘤(ENKL)是一种罕见的成熟T细胞和NK 细胞淋巴瘤亚型,与EB病毒相关。病例:一名20岁患者因严重神经系统症状就诊,确诊为IV期鼻型ENKL并累及中枢神经系统(CNS)。患者总计接受了9线治疗,包括化疗、全脑全脊髓照射、异基因干细胞移植(alloSCT)、供者淋巴细胞输注,以及新型药物(纳武利尤单抗、达雷妥尤单抗、沙利度胺、来那度胺、病毒特异性T细胞)联合鞘内化疗。治疗效果通过血液和脑脊液(CSF)检查进行评估。一线SMILE化疗使全身和CNS病变缓解。随后,阿糖胞苷为基础的化疗联合达雷妥尤单抗,在alloSCT前再次诱导缓解。

疗效监测应同时包括血液和CSF分析。大剂量阿糖胞苷为基础的化疗联合达雷妥尤单抗和鞘内化疗,可考虑作为针对CNS的挽救治疗。本文为现有有限证据补充了资料,提示达雷妥尤单抗能够穿过血脑屏障。

展开英文摘要原文

Background: Extranodal natural killer/T-cell lymphoma (ENKL) is a rare subtype of mature T and natural killer cell lymphomas associated with Epstein-Barr virus. Case: A 20-year-old presented with severe neurological symptoms and was diagnosed with stage IV ENKL, nasal type, with CNS involvement.

Overall, the patient received nine treatment lines, including chemotherapy, craniospinal irradiation, allogeneic stem cell transplant (alloSCT), donor lymphocyte infusions, and novel agents (Nivolumab, Daratumumab, Thalidomide, Lenalidomide, virus-specific T cells) combined with intrathecal chemotherapy. The treatment effect was evaluated in both blood and CSF (cerebrospinal fluid).

First-line SMILE chemotherapy resulted in systemic and CNS remission. Later Cytarabine-based chemotherapy and Daratumumab combination helped to reinduce remission before alloSCT. Conclusion: We show that efficacy monitoring should include both blood and CSF analysis. High-dose Cytarabine-based chemotherapy in combination with Daratumumab and intrathecal chemotherapy may be considered as salvage CNS-directed therapies.

We add to existing limited data that Daratumumab penetrates the blood-brain barrier.

论文信息

作者
Dapkevičiūtė-Purlienė A、Augustinavičius V、Žučenka A
单位
Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, Vilnius, Lithuania.
文献类型
病例报告
期刊
Pathology oncology research : POR2022
原文标识
PubMed 36699620 · DOI 10.3389/pore.2022.1610866