title: Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome.
abstract: Bing Neel syndrome is a rare disease manifestation of Waldenström's macroglobulinemia that results from infiltration of the central nervous system by malignant lymphoplasmacytic cells. In this guideline we describe the clinical symptoms, as well as the appropriate laboratory and radiological studies, that can aid in the diagnosis. The presentation of Bing Neel syndrome may be very diverse, and includes headaches, cognitive deficits, paresis, and psychiatric symptoms. The syndrome can present in patients with known Waldenström's macroglobulinemia, even in the absence of systemic progression, but also in previously undiagnosed patients. Diagnostic work-up should include cerebral spinal fluid analysis with multiparameter flow cytometry to establish B-cell clonality, protein electrophoresis and immunofixation for the detection and classification of a monoclonal protein as well as molecular diagnostic testing for immunoglobulin gene rearrangement and mutated MYD88. MRI of the brain and spinal cord is also essential. The second challenge is to expand our knowledge of prognosis and treatment outcome. Prospective clinical trials on Bing Neel syndrome patients that employ uniform treatment along with appropriate laboratory cerebral spinal fluid assessments and standardized MRI protocols will be invaluable, constituting a significant step forward in delineating treatment outcome for this intriguing disease manifestation.
authors.affiliation: Department of Hematology, UMC Utrecht Cancer Center, the Netherlands m.c.minnema@umcutrecht.nl.
authors.forename: Monique C
authors.initials: MC
authors.lastname: Minnema
authors.affiliation: Hematology Center, Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Eva
authors.initials: E
authors.lastname: Kimby
authors.affiliation: Cancer Division, University College London Hospitals NHS Foundation Trust, UK.
authors.forename: Shirley
authors.initials: S
authors.lastname: D'Sa
authors.affiliation: Department of Oncology and Hematology, Hôpital Universitaires de Strasbourg and Université de Strasbourg, France.
authors.forename: Luc-Matthieu
authors.initials: LM
authors.lastname: Fornecker
authors.affiliation: Service d'Hématologie-Immunologie-Cytogénétique, Centre Hospitalier de Valenciennes/Laboratoire d'Hématologie, Centre de Biologie et Pathologie, CHRU de Lille/INSERM, France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Poulain
authors.affiliation: Department of Neurology & Neurosurgery, Brain Center Rudolf Magnus, UMC Utrecht, The Netherlands.
authors.forename: Tom J
authors.initials: TJ
authors.lastname: Snijders
authors.affiliation: Department of Clinical Therapeutics, National and Kapodistrian University of Athens, Greece.
authors.forename: Efstathios
authors.initials: E
authors.lastname: Kastritis
authors.affiliation: Pôle d'Imagerie-Neuroradiologie, Hôpital de Hautepierre/CHU Strasbourg, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Kremer
authors.affiliation: Pôle d'Imagerie-Neuroradiologie, Hôpital de Hautepierre/CHU Strasbourg, France.
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Fitsiori
authors.affiliation: Department of Oncology and Hematology, Hôpital Universitaires de Strasbourg and Université de Strasbourg, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Simon
authors.affiliation: Laboratory of Hematology, Hôpital Pitié Salpêtrière, Paris, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Davi
authors.affiliation: Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
authors.forename: Michael
authors.initials: M
authors.lastname: Lunn
authors.affiliation: Bing Center for Waldenstrom's Macroglobulinemia, Dana Farber Cancer Institute and Harvard Medical; School, Boston, MA, USA.
authors.forename: Jorge J
authors.initials: JJ
authors.lastname: Castillo
authors.affiliation: Bing Center for Waldenstrom's Macroglobulinemia, Dana Farber Cancer Institute and Harvard Medical; School, Boston, MA, USA.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Patterson
authors.affiliation: Laboratory of Hematology, Hôpital Pitié Salpêtrière, Paris, France.
authors.forename: Magali
authors.initials: M
authors.lastname: Le Garff-Tavernier
authors.affiliation: Laboratory of Hematology, Hôpital Pitié Salpêtrière, Paris, France.
authors.forename: Myrto
authors.initials: M
authors.lastname: Costopoulos
authors.affiliation: AP-HP Hôpital Pitié Salpêtrière, UPMC univ Paris, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Leblond
authors.affiliation: Department of Hematology, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Marie-José
authors.initials: MJ
authors.lastname: Kersten
authors.affiliation: Department of Clinical Therapeutics, National and Kapodistrian University of Athens, Greece.
authors.forename: Meletios A
authors.initials: MA
authors.lastname: Dimopoulos
authors.affiliation: Bing Center for Waldenstrom's Macroglobulinemia, Dana Farber Cancer Institute and Harvard Medical; School, Boston, MA, USA.
authors.forename: Steven P
authors.initials: SP
authors.lastname: Treon
mesh_terms: D000465:Algorithms; D003131:Combined Modality Therapy / Q000009:adverse effects / Q000379:methods; D003937:Diagnosis, Differential; D003955:Diagnostic Tests, Routine; D019468:Disease Management; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D025202:Molecular Diagnostic Techniques; D010641:Phenotype; D013577:Syndrome; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy*