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布鲁顿氏酪氨酸激酶抑制剂治疗原发中枢神经系统淋巴瘤16例临床分析

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目的:分析应用布鲁顿氏酪氨酸激酶(BTK)抑制剂治疗原发中枢神经系统淋巴瘤(PCNSL)患者的临床特征,探讨影响疗效的因素及疗效不佳患者的后续治疗经验.方法:回顾性分析初诊应用BTK抑制剂(包括奥布替尼、泽布替尼)联合传统化疗诱导治疗的PCNSL患者的临床资料.结果:16例PCNSL患者中,男性13例,女性3例,中位年龄62.5岁.病灶数目以多发为主,占56%;病理分型以非生发中心B细胞(non-GCB)型为主,占62%;双表达型占38%;9例患者有初诊脑脊液生化数据,脑脊液蛋白升高者占78%;7例患者有基因测序结果,MYD88突变占57%,CD79B突变占43%;16例患者中9例(56%)接受奥布替尼联合传统化疗治疗,7例(44%)接受泽布替尼联合传统化疗治疗;16例患者的总体反应率(ORR)为69%.5例疗效不佳的患者中,4例为多发病灶,4例为双表达类型,3例具有MYD88突变;2例接受造血干细胞移植序贯嵌合抗原受体T细胞治疗(auto-HSCT+CAR-T),获得完全缓解.结论:BTK抑制剂联合传统化疗治疗PCNSL患者的ORR为69%,多发病灶、双表达、MYD88突变可能是疗效差的因素.auto-HSCT+CAR-T可能是BTK抑制剂联合传统化疗治疗PCNSL疗效不佳患者可行的后续治疗方案.
Bruton's tyrosine kinase inhibitor for primary central nervous system lymphoma:a clinical analysis of 16 cases
Objective:To analyze the clinical characteristics of patients with primary central nervous system lymphoma(PCNSL)treated with Bruton's tyrosine kinase(BTK)inhibitors and to explore the factors affecting the efficacy and the fol-low-up treatment experience of patients with poor efficacy.Methods:The clinical data of newly diagnosed PCNSL patients treated with BTK inhibitors(including orelabrutinib,zanubrutinib)combined with conventional chemotherapy were retro-spectively analyzed.Results:Among the 16 patients with PCNSL,13 were males and 3 were females,with a median age of 62.5 years.The lesions were mainly multiple,accounting for 56%.The main pathological type was non-germinal center B cell(62%),and double expression type(38%).The biochemical data of cerebrospinal fluid were found in 9 patients,and the protein level of cerebrospinal fluid was elevated in 78%.A total of 7 patients had gene sequencing results,including MYD88 mutation(57%)and CD79B mutation(43%).Among the 16 patients,9(56%)received orelabrutinib combined with conventional chemotherapy,and 7(44%)received zanubrutinib combined with conventional chemotherapy.The overall response rate(ORR)of the 16 patients was 69%.Among the 5 patients with poor efficacy,4 had multiple lesions,4 had double expression type,and 3 had MYD88 mutation.Two patients underwent hematopoietic stem cell transplantation followed by chimeric antigen receptor T cell therapy(auto-HSCT+CAR-T)and achieved complete remission.Conclusion:The ORR of PCNSL patients treated with BTK inhibitor combined with conventional chemotherapy is 69%.Multiple lesions,double expression and MYD88 mutation may be the factors of poor efficacy.The auto-HSCT+CAR-T may be a feasible follow-up treatment for patients with poor response to PCNSL treated by BTK inhibitors combined with conventional chemotherapy.

Primary central nervous system lymphomaBruton's tyrosine kinase inhibitorsClinical features

戴紫港、周晓曦、张义成、黄亮

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华中科技大学同济医学院附属同济医院血液内科,湖北武汉 430030

原发中枢神经系统淋巴瘤 布鲁顿氏酪氨酸激酶抑制剂 临床特征

2024

内科急危重症杂志
华中科技大学同济医学院

内科急危重症杂志

CSTPCD
影响因子:0.947
ISSN:1007-1024
年,卷(期):2024.30(2)
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