Bing-Neel syndrome successfully treated with venetoclax and zanubrutinib

Rationale: Bing-Neel syndrome (BNS) is an uncommon neurological manifestation of Waldenström’s Macroglobulinemia (WM). The diagnosis of BNS is still challenging due to its low incidence and high heterogeneity. A definite diagnosis relies on histopathology and cerebrospinal fluid (CSF) analysis to identify lymphoplasmacytic lymphocyte infiltration of the central nervous system. MYD88 L265P mutation also serves as a valuable diagnostic tool. To date, there is no consensus on the standard management of BNS, and current evidence is largely limited to reports on the use of high-dose chemotherapy and BTK inhibitors. Herein, we present a case of BNS who achieved remission following treatment with venetoclax and zanubrutinib. Furthermore, venetoclax was detected in CSF. Patient concern: A 66-year-old female with WM presenting with a headache. Diagnoses: Bing-Neel syndrome. Interventions: The patient was diagnosed with BNS through CSF examination and treated with zanubrutinib plus venetoclax. Outcomes: The patient’s symptoms resolved, and abnormal B lymphocytes were not detected in CSF. Lesson: BNS is an uncommon manifestation of WM, with significant diagnostic challenges. In the absence of standard management for BNS, high-dose chemotherapy and BTK inhibitors are widely used as front-line approaches. BCL-2 inhibitors, which have shown notable CNS-penetrating capacity, hold considerable promise for the treatment of BNS and merit further exploration.