Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
Br J Haematol. 2019 Nov;187(3):277-285. doi: 10.1111/bjh.16167. Epub 2019 Aug 20.
Bing-Neel syndrome (BNS) is an uncommon presentation of Waldenström macroglobulinaemia (WM), seen during the course of the disease in about 1% of patients. BNS occurs when WM cells gain access to the central nervous system (CNS) causing neurological deficits. The diagnosis of BNS is suggested by the presence of radiological abnormalities, such as leptomeningeal enhancement on magnetic resonance imaging and confirmed by the presence of clonal lymphoplasmacytic cells and MYD88 L265P in the cerebrospinal fluid. The treatment of BNS requires agents with good penetration into the CNS, such as fludarabine, methotrexate and cytarabine. The novel Bruton Tyrosine Kinase inhibitor ibrutinib has shown CNS-penetrating properties, and recent data suggest a therapeutic role in BNS. In this review, we will discuss the clinical and pathological features, diagnostic criteria, treatment options and outcomes of patients with BNS.
宾-尼尔综合征（BNS）是瓦尔登斯特伦巨球蛋白血症（WM）的一种罕见表现，在疾病过程中约有 1%的患者出现。当 WM 细胞进入中枢神经系统（CNS）导致神经功能缺损时，就会发生 BNS。BNS 的诊断依据是存在放射学异常，如磁共振成像上的软脑膜增强，并通过脑脊液中存在克隆性淋巴浆细胞和 MYD88 L265P 得到证实。BNS 的治疗需要具有良好 CNS 穿透性的药物，如氟达拉滨、甲氨蝶呤和阿糖胞苷。新型布鲁顿酪氨酸激酶抑制剂伊布替尼具有 CNS 穿透性，最近的数据表明其在 BNS 中有治疗作用。在这篇综述中，我们将讨论 BNS 患者的临床和病理特征、诊断标准、治疗选择和结局。
