混合扩散和瘤形式的宾尼尔综合征成功地用易布鲁替尼治疗
Junid A Naveed Ahmad1, Brett A Schroeder1,2, John Paul T Yun3
1Virginia Mason Medical Center, Cancer Institute, Seattle, WA, USA.
Case reports in oncology
|November 10, 2023
概括
宾尼尔综合征 (BNS) 是一种罕见的中枢神经系统并发症,是沃尔登斯特罗姆巨型球蛋白血症 (WM) 的一种罕见的并发症,可以用布鲁顿氨酸激酶抑制剂 (BTKIs) 有效治疗. 本案例研究突出了一个患者,他通过IBRUTINIB (BTKI) 实现了显著的临床和放射性改善,证明了IBRUTINIB对BNS的治疗潜力.
科学领域:
- 神经学 神经学
- 血液学 血液学 血液学
- 在瘤学瘤学.
背景情况:
- 宾尼尔综合征 (Bing-Neel syndrome,简称BNS) 是一种罕见的中枢神经系统 (中枢神经系统) 症状,表现为沃尔登斯特罗姆巨型球蛋白血症 (WM).
- BNS涉及恶性淋巴细胞细胞透到中枢神经系统,导致各种神经症状.
- 最近,BNS的标准化诊断标准和治疗选择得到了改进.
研究的目的:
- 介绍一个成功用布鲁顿氨酸激酶抑制剂 (BTKI) 治疗的Bing-Neel综合征病例.
- 讨论MYD88 L265P突变测试在BNS诊断中的作用.
- 突出BTKI在管理BNS中的不断扩大的治疗潜力.
主要方法:
- 一个70岁的男性病例介绍,他有WM病史,有神经症状.
- 诊断工作包括脑部MRI,血清和脑脊液研究,以及立体脑部活检.
- 治疗包括每天口服布鲁丁尼布 (BTKI).
主要成果:
- 大脑活检证实淋巴细胞淋巴瘤与MYD88 L265P突变.
- 患者对ibrutinib有显著的临床和放射性反应.
- 这些积极的反应已经持续了超过31个月.
结论:
- 布鲁顿氨酸激酶抑制剂 (BTKI) 具有很高的中枢神经系统透性,并为BNS提供了一种新的治疗策略.
- 用BTKI有效治疗BNS可以改善患者的生存率和生活质量.
- 在BNS管理的背景下,MYD88 L265P突变测试至关重要.
关键词:
宾尼尔综合征 (Bing-Neel综合征) 是一种布鲁顿氨酸激酶抑制剂 布鲁顿氨酸激酶抑制剂淋巴质细胞性淋巴瘤是淋巴细胞性淋巴瘤.MYD88 L265P 在线阅读沃尔登斯特罗姆的巨型球蛋白血症更多相关视频
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