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.

1st Department of Medicine 1st Faculty of Medicine Charles University and General University Hospital Prague Czech Republic

Amsterdam University Medical Centers Cancer Center Amsterdam University of Amsterdam Amsterdam The Netherlands

Bing Center for Waldenström's Macroglobulinemia Dana Farber Cancer Institute Harvard Medical School Boston MA

Centre for Waldenström Macroglobulinaemia and Related Conditions University College London Hospitals NHS Foundation Trust London UK

Colorado Blood Cancer Institute Sarah Cannon Research Institute Denver CO

Department of Hematology University Medical Center Utrecht Utrecht University Utrecht the Netherlands

Division of Hematology department of Medicine Karolinska Institutet Huddinge Stockholm Sweden

Hematology Department Sorbonne University Pitié Salpêtrière hospital Paris France

Hematology Division Department of Molecular Biotechnologies and Health Sciences University of Torino Torino Italy

Hematology Division National Hospital Organization Disaster Medical Center Tokyo Japan

Institute of Experimental Cancer Research University Hospital Ulm Ulm Germany

Massachusetts General Hospital Cancer Center Boston MA

State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood Diseases Haihe Laboratory of Cell Ecosystem Institute of Hematology and Blood Diseases Hospital Chinese Academy of Medical Sciences and Peking Union Medical College China and Tianjin Institutes of Health Science Tianjin China

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