Overview
Bing-Neel syndrome (BNS) is a rare complication characterized by lymphomatous infiltration of the central nervous system (CNS) in patients with Waldenström macroglobulinemia (WM), leading to neurological symptoms and often radiological abnormalities 1.Diagnosis
Radiologic and cytologic confirmation of CNS involvement 1.
Symptoms indicative of CNS involvement, such as neurological deficits or altered mental status 1.
Cerebrospinal fluid (CSF) analysis showing lymphomatous cells 1.Management
First-line treatment: Ibrutinib monotherapy is increasingly used, with dosing typically at 560 mg or 420 mg once daily 1.
Response criteria: Evaluated based on improvement or resolution of symptoms, radiologic abnormalities, and clearance of disease in CSF 1.
Efficacy: Symptomatic and radiologic improvements observed in 85% and 60% of patients within 3 months, respectively 1.
Best response: 85% improvement/resolution of symptoms, 83% improvement/resolution of radiologic abnormalities, and 47% CSF clearance 1.Special Populations
Elderly: No specific data provided; however, the median age at BNS diagnosis was 65 years, suggesting relevance to elderly patients 1.
Comorbidities: Management considerations for comorbidities not explicitly detailed in the provided abstracts 1.Key Recommendations
Consider ibrutinib monotherapy as a first-line treatment for BNS, with dosing options of 560 mg or 420 mg once daily (Evidence: Moderate) 1.
Monitor response using criteria that include symptomatic improvement, radiologic resolution, and CSF clearance (Evidence: Moderate) 1.
Evaluate patients closely for potential discontinuation due to toxicity, progression, or death within 2 years of therapy initiation (Evidence: Weak) 1.References
1 Castillo JJ, Itchaki G, Paludo J, Varettoni M, Buske C, Eyre TA et al.. Ibrutinib for the treatment of Bing-Neel syndrome: a multicenter study. Blood 2019. link