Drozd-Sokołowska Joanna, Waszczuk-Gajda Anna, Witkowska Magdalena, Sienkiewicz Elżbieta, Kopińska Anna, Kołkowska-Leśniak Agnieszka, Barankiewicz Joanna, Długosz-Danecka Monika, Smolewski Piotr, Helbig Grzegorz, Lech-Marańda Ewa, Jurczak Wojciech, Biecek Przemysław, Giebel Sebastian, Wiktor-Jędrzejczak Wiesław, Basak Grzegorz
Department of Hematology, Transplantation and Internal Medicine, Medical University of Warsaw, Banacha 1a Str., 02-097 Warsaw, Poland.
Department of Experimental Hematology, Medical University of Lodz, 93-510 Lodz, Poland.
J Clin Med. 2022 Jul 30;11(15):4447. doi: 10.3390/jcm11154447.
Bing-Neel syndrome (BNS) is a rare presentation of Waldenström macroglobulinemia (WM). BNS is a consequence of the central nervous system (CNS) involvement by lymphoplasmacytic lymphoma (LPL) and, rarely, the peripheral nervous system. The data on BNS are extremely scarce. Therefore, we performed a multicenter retrospective analysis of BNS patients diagnosed and treated in centers aligned with the Polish Lymphoma Research Group. The analysis covers the years 2014-2021. Eleven patients were included, 55% females and the median age at BNS diagnosis was 61 years. The median time from WM to BNS was 3.5 years; 27% of patients did have a diagnosis of WM and BNS made simultaneously or within 30 days from each other. Isolated parenchymal involvement was the least frequent (20%). Patients were treated with different regimens, mostly able to cross the blood-brain barrier, including 18% treated with ibrutinib first line. The cumulative objective response to treatment was 73%. With the median follow-up of 20 months (95% CI, 2-32), the 36-month estimates were: overall survival (OS) 47%, progression-free survival (PFS) 33%, and cumulative incidence of BNS-associated death 41%. The performance status according to ECOG was significant for PFS (HR = 7.79) and the hemoglobin concentration below 11 g/dL was correlated with PFS. To conclude, BNS is a very rare manifestation of WM. It is associated with a poor outcome with most patients succumbing to BNS.
宾-尼尔综合征（BNS）是华氏巨球蛋白血症（WM）的一种罕见表现形式。BNS是淋巴浆细胞淋巴瘤（LPL）累及中枢神经系统（CNS）的结果，很少累及外周神经系统。关于BNS的数据极为稀少。因此，我们对波兰淋巴瘤研究小组下属各中心诊断和治疗的BNS患者进行了多中心回顾性分析。该分析涵盖2014年至2021年。共纳入11例患者，其中55%为女性，BNS诊断时的中位年龄为61岁。从WM到BNS的中位时间为3.5年；27%的患者WM和BNS诊断同时进行或间隔在30天内。孤立性实质受累最为少见（20%）。患者接受了不同的治疗方案，多数方案能够通过血脑屏障，其中18%的患者一线接受伊布替尼治疗。治疗的累积客观缓解率为73%。中位随访20个月（95%CI，2 - 32），36个月的估计结果为：总生存期（OS）47%，无进展生存期（PFS）33%，BNS相关死亡的累积发生率41%。根据东部肿瘤协作组（ECOG）标准评估的体能状态对PFS有显著影响（HR = 7.79），血红蛋白浓度低于11 g/dL与PFS相关。总之，BNS是WM一种非常罕见的表现形式。其预后较差，大多数患者死于BNS。
