title: Bing-Neel syndrome, a rare complication of Waldenström macroglobulinemia: analysis of 44 cases and review of the literature. A study on behalf of the French Innovative Leukemia Organization (FILO).
abstract: Central nervous system involvement by malignant cells is a rare complication of Waldenström macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, therapeutic approaches and response evaluation for this syndrome. In this series, we retrospectively analyzed 44 French patients with Bing-Neel syndrome. Bing-Neel syndrome was the first manifestation of Waldenström macroglobulinemia in 36% of patients. When Waldenström macroglobulinemia was diagnosed prior to Bing-Neel syndrome, the median time interval between this diagnosis and the onset of Bing-Neel syndrome was 8.9 years. This study highlights the possibility of the occurrence of Bing-Neel syndrome without any other evidence of progression of Waldenström macroglobulinemia. The clinical presentation was heterogeneous without any specific signs or symptoms. Biologically, the median lymphocyte count in the cerebrospinal fluid was 31/mm(3). Magnetic resonance imaging revealed abnormalities in 78% of the cases. The overall response rate after first-line treatment was 70%, and the overall survival rate after the diagnosis of Bing-Neel syndrome was 71% at 5 years. Altogether, these results suggest that Bing-Neel syndrome should be considered in the context of any unexplained neurological symptoms associated with Waldenström macroglobulinemia. The diagnostic approach should be based on cerebrospinal fluid analysis and magnetic resonance imaging of the brain and spinal axis. It still remains difficult to establish treatment recommendations or prognostic factors in the absence of large-scale, prospective, observational studies.
authors.affiliation: Department of Oncology and Hematology, Hôpitaux Universitaires de Strasbourg and Université de Strasbourg, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Simon
authors.affiliation: Department of Radiology, Hôpitaux Universitaires de Strasbourg and Université de Strasbourg, France.
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Fitsiori
authors.affiliation: Department of Cell Therapy and Clinical Hematology, Centre Hospitalier Universitaire, Clermont-Ferrand, France.
authors.forename: Richard
authors.initials: R
authors.lastname: Lemal
authors.affiliation: Lymphoid Malignancies Unit, Hôpital Henri Mondor, AP-HP, Créteil, France.
authors.forename: Jehan
authors.initials: J
authors.lastname: Dupuis
authors.affiliation: Department of Hematology, Hôpital Claude Huriez, Lille, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Carpentier
authors.affiliation: Department of Medical Oncology, Hôpital d'Instruction des Armées, Toulon, France.
authors.forename: Laurys
authors.initials: L
authors.lastname: Boudin
authors.affiliation: Department of Hematology, Centre Hospitalier Universitaire, Angers, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Corby
authors.affiliation: Department of Hematology, Institut Paoli-Calmettes, Marseille, France.
authors.forename: Thérèse
authors.initials: T
authors.lastname: Aurran-Schleinitz
authors.affiliation: Department of Onco-Hematology, Centre Antoine Lacassagne, Nice, France.
authors.forename: Lauris
authors.initials: L
authors.lastname: Gastaud
authors.affiliation: Department of Clinical Immunology, Hôpital Saint-Louis, AP-HP and Université Paris Diderot, Sorbonne Paris Cité, France.
authors.forename: Alexis
authors.initials: A
authors.lastname: Talbot
authors.affiliation: Department of Hematology, Centre Henri Becquerel, Rouen, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Leprêtre
authors.affiliation: Department of Hematology, Centre Hospitalier Universitaire, Nantes, France.
authors.forename: Béatrice
authors.initials: B
authors.lastname: Mahe
authors.affiliation: Department of Hematology, Centre Hospitalier Universitaire, Besançon, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Payet
authors.affiliation: Department of Hematology, Institut Curie-Hôpital René Huguenin, Saint-Cloud, France.
authors.forename: Carole
authors.initials: C
authors.lastname: Soussain
authors.affiliation: Department of Neurosurgery, Centre Hospitalier Universitaire, Bordeaux, France.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Bonnet
authors.affiliation: Department of Hematology, Centre Hospitalier Universitaire, Montpellier, France.
authors.forename: Laure
authors.initials: L
authors.lastname: Vincent
authors.affiliation: Department of Hematology, Centre Hospitalier Universitaire, Tours, France.
authors.forename: Séverine
authors.initials: S
authors.lastname: Lissandre
authors.affiliation: Department of Oncology and Hematology, Hôpitaux Universitaires de Strasbourg and Université de Strasbourg, France.
authors.forename: Raoul
authors.initials: R
authors.lastname: Herbrecht
authors.affiliation: Department of Radiology, Hôpitaux Universitaires de Strasbourg and Université de Strasbourg, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Kremer
authors.affiliation: Department of Hematology, APHP Hôpital Pitié-Salpêtrière, UPMC Paris, GRECHY, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Leblond
authors.affiliation: Department of Oncology and Hematology, Hôpitaux Universitaires de Strasbourg and Université de Strasbourg, France luc-matthieu.fornecker@chru-strasbourg.fr.
authors.forename: Luc-Matthieu
authors.initials: LM
authors.lastname: Fornecker
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D016543:Central Nervous System Neoplasms / Q000134:cerebrospinal fluid / Q000401:mortality / Q000473:pathology / Q000556:secondary / Q000628:therapy; D005260:Female; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D008258:Waldenstrom Macroglobulinemia / Q000134:cerebrospinal fluid / Q000401:mortality / Q000473:pathology / Q000628:therapy