title: Bing-Neel syndrome: Two unexpected cases and a review of the literature.
abstract: Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma characterized by the proliferation of small B-lymphocytes in the bone marrow that produce monoclonal immunoglobulin M (IgM). We describe two patients with WM who presented with neurological symptoms due to infiltration of lymphoplasmacytoid tumor cells in the central nervous system, a condition known as Bing-Neel syndrome. A literature review revealed that this syndrome is rare and commonly missed in clinical practice due to its variable presentation and a lack of awareness or knowledge. Brain and spinal magnetic resonance imaging may show a focal mass or diffuse infiltration. The diagnosis of Bing-Neel syndrome requires proof of IgM or lymphoplasmacytoid cells in cerebrospinal fluid or in a brain biopsy. Treatment with intravenous and/or intrathecal chemotherapy and cranial radiotherapy is described in literature with generally poor outcome, although a combination of these therapies seems to improve outcome. Nevertheless, insufficient data are currently available to make general treatment recommendations.
authors.affiliation: Department of Neurology, University Clinic RWTH Aachen, Germany; Department of Neurology, AZ Sint Jan Brugge Oostende, Belgium. Electronic address: margot.vancauwenberge@gmail.com.
authors.forename: M G
authors.initials: MG
authors.lastname: Van Cauwenberge
authors.affiliation: Department of Laboratory Hematology, University Hospital Ghent, Belgium.
authors.forename: B
authors.initials: B
authors.lastname: Depreter
authors.affiliation: Department of Laboratory Hematology, University Hospital Ghent, Belgium.
authors.forename: E N
authors.initials: EN
authors.lastname: Dumoulin
authors.affiliation: Department of Laboratory Hematology, AZ Sint Jan Brugge Oostende, Belgium.
authors.forename: J
authors.initials: J
authors.lastname: Emmerechts
authors.affiliation: Department of Laboratory Hematology, AZ Sint Jan Brugge Oostende, Belgium.
authors.forename: F
authors.initials: F
authors.lastname: Nollet
authors.affiliation: Department of Neurology, AZ Sint Jan Brugge Oostende, Belgium.
authors.forename: L J
authors.initials: LJ
authors.lastname: Vanopdenbosch
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001402:B-Lymphocytes / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D004358:Drug Therapy; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology / Q000473:pathology; D007075:Immunoglobulin M / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male; D009154:Mutation / Q000235:genetics; D053594:Myeloid Differentiation Factor 88 / Q000235:genetics / Q000378:metabolism; D011878:Radiotherapy; D013116:Spinal Cord / Q000473:pathology; D008258:Waldenstrom Macroglobulinemia / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology* / Q000628:therapy*
chemical_list: D007075:Immunoglobulin M; C507779:MYD88 protein, human; D053594:Myeloid Differentiation Factor 88
keywords: Bing–Neel syndrome; Central nervous system lymphoma; Intrathecal chemotherapy; Lymphoplasmacytic lymphoma; MYD88; Radiotherapy; Waldenström macroglobulinemia