Sarosiek Shayna, Becking Anne-Marie L, Branagan Andrew, Ferrero Simone, Khwaja Jahanzaib, Kimby Eva, Roos-Weil Damien, Sekiguchi Naohiro, Trneny Marek, Yi Shuhua, Patterson Christopher J, Buske Christian, Matous Jeffrey V, Treon Steven P, Minnema Monique C
Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
Amsterdam University Medical Centers, Cancer Center Amsterdam, University of Amsterdam, Amsterdam, The Netherlands.
Semin Hematol. 2025 Apr;62(2):85-89. doi: 10.1053/j.seminhematol.2025.04.005. Epub 2025 Apr 16.
Consensus panel 2 from the 12th International Workshop on Waldenstrom Macroglobulinemia was tasked with updating the guidelines on the diagnosis and management of patients with Bing-Neel syndrome (BNS). In this panel we have summarized the clinical symptoms that may be present with BNS, discussed the criteria required for diagnosis of BNS, made recommendations for follow-up imaging, and proposed revised guidelines for response assessment in BNS. The key recommendations from the 12th International Workshop on WM (IWWM-12) Consensus panel 2 include: (1) the establishment of zanubrutinib as a standard therapy for treatment of BNS; (2) recommendations on imaging and CSF evaluation during treatment and follow-up of BNS; and (3) revised response criteria in view of new data showing that malignant cells can persist in the CSF of many patients treated with BTK-inhibitors. New categorical response categories proposed include that for a Clinical Complete Response and Progressive Disease.
第12届华氏巨球蛋白血症国际研讨会的共识小组2负责更新宾-尼尔综合征（BNS）患者的诊断和管理指南。在本小组中，我们总结了BNS可能出现的临床症状，讨论了BNS的诊断标准，对随访影像学检查提出了建议，并提出了BNS反应评估的修订指南。第12届华氏巨球蛋白血症国际研讨会（IWWM-12）共识小组2的主要建议包括：（1）确立泽布替尼作为BNS治疗的标准疗法；（2）关于BNS治疗和随访期间影像学检查和脑脊液评估的建议；（3）鉴于新数据显示许多接受BTK抑制剂治疗的患者脑脊液中恶性细胞可能持续存在，对反应标准进行修订。新提出的分类反应类别包括临床完全缓解和疾病进展。
