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Report of Consensus Panel 2 from the 12th International Workshop on the management of Bing-Neel syndrome in patients with Waldenstrom's Macroglobulinemia

S. Sarosiek, AL. Becking, A. Branagan, S. Ferrero, J. Khwaja, E. Kimby, D. Roos-Weil, N. Sekiguchi, M. Trneny, S. Yi, CJ. Patterson, C. Buske, JV. Matous, SP. Treon, MC. Minnema

. 2025 ; 62 (2) : 85-89. [pub] 20250416

Language English Country United States

Document type Consensus Development Conference, Journal Article

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.

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$a Consensus panel 2 from the 12th International Workshop on Waldenstrom Macroglobulinemia was tasked with updating the guidelines on the diagnosis and management of patients with Bing-Neel syndrome (BNS). In this panel we have summarized the clinical symptoms that may be present with BNS, discussed the criteria required for diagnosis of BNS, made recommendations for follow-up imaging, and proposed revised guidelines for response assessment in BNS. The key recommendations from the 12th International Workshop on WM (IWWM-12) Consensus panel 2 include: (1) the establishment of zanubrutinib as a standard therapy for treatment of BNS; (2) recommendations on imaging and CSF evaluation during treatment and follow-up of BNS; and (3) revised response criteria in view of new data showing that malignant cells can persist in the CSF of many patients treated with BTK-inhibitors. New categorical response categories proposed include that for a Clinical Complete Response and Progressive Disease.
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$a Becking, Anne-Marie L $u Amsterdam University Medical Centers, Cancer Center Amsterdam, University of Amsterdam, Amsterdam, The Netherlands
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$a Branagan, Andrew $u Massachusetts General Hospital Cancer Center, Boston MA
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$a Ferrero, Simone $u Hematology Division, Department of Molecular Biotechnologies and Health Sciences, University of Torino, Torino, Italy
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$a Khwaja, Jahanzaib $u Centre for Waldenström Macroglobulinaemia and Related Conditions, University College London Hospitals NHS Foundation Trust, London, UK
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$a Kimby, Eva $u Division of Hematology, department of Medicine, Karolinska Institutet Huddinge, Stockholm, Sweden
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$a Patterson, Christopher J $u Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA
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