Bing-Neel syndrome is a rare CNS manifestation of Waldenstrom's macroglobulinemia in which lymphoplasmacytic cells migrate into the central nervous system. When symptomatic, it requires active management.
Bing-Neel syndrome with neurologic deficits — including headaches, seizures, cranial nerve palsies, weakness in limbs, and atypical neuropathy — with definitive diagnosis confirmed by the presence of clonal B-cells in cerebrospinal fluid or cerebral tissue biopsy.
For symptomatic Bing-Neel syndrome, systemic therapy is recommended, with a targeted BTK inhibitor-based approach as the preferred class. The complete regimen, full list of alternatives, and options for specific circumstances are detailed in the full protocol.
DOI: 10.6004/jnccn.2024.0001
Bing-Neel syndrome (BNS) is a rare manifestation of WM that results in the migration of lymphoplasmacytic cells to the CNS.
Neurologic deficits concerning BNS include but are not limited to headaches, seizures, cranial nerve palsies, weakness in limbs, and atypical neuropathy.
Definitive diagnosis of BNS includes presence of clonal B-cells in CSF or within a tissue biopsy with a typical manifestation and presentation of systemic disease.
If a person is symptomatic, various systemic therapy options are recommended; preferred regimens include BTK inhibitors such as ibrutinib and zanubrutinib.
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