@article{Laurence Simon_Aikaterini Fitsiori_Richard Lemal_Jehan Dupuis_Benjamin Carpentier_Laurys Boudin_Anne Corby_Thérèse Aurran-Schleinitz_Lauris Gastaud_Alexis Talbot_Stéphane Leprêtre_Béatrice Mahe_Camille Payet_Carole Soussain_Charlotte Bonnet_Laure Vincent_Séverine Lissandre_Raoul Herbrecht_Stéphane Kremer_Véronique Leblond_Luc-Matthieu Fornecker_2015, place={Pavia, Italy}, 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).}, volume={100}, url={https://haematologica.org/article/view/7587}, DOI={10.3324/haematol.2015.133744}, abstractNote={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<sup>3</sup&gt;. 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.}, number={12}, journal={Haematologica}, author={Laurence Simon and Aikaterini Fitsiori and Richard Lemal and Jehan Dupuis and Benjamin Carpentier and Laurys Boudin and Anne Corby and Thérèse Aurran-Schleinitz and Lauris Gastaud and Alexis Talbot and Stéphane Leprêtre and Béatrice Mahe and Camille Payet and Carole Soussain and Charlotte Bonnet and Laure Vincent and Séverine Lissandre and Raoul Herbrecht and Stéphane Kremer and Véronique Leblond and Luc-Matthieu Fornecker}, year={2015}, month={Nov.}, pages={1587-1594} }