Bing-Neel syndrome, a rare complication of Waldenstrom's macroglobulinemia: analysis of 44 cases and review of the literature. A study on behalf of the French Innovative Leukemia Organization (FILO).
Fiche publication
Date publication
septembre 2015
Auteurs
Membres identifiés du Cancéropôle Est :
Pr HERBRECHT Raoul
Tous les auteurs :
Simon L, Fitsiori A, Lemal R, Dupuis J, Carpentier B, Boudin L, Corby A, Aurran-Schleinitz T, Gastaud L, Talbot A, Lepretre S, Mahe B, Payet C, Soussain C, Bonnet C, Vincent L, Lissandre S, Herbrecht R, Kremer S, Leblond V, Fornecker LM
Lien Pubmed
Résumé
Central nervous system involvement by malignant cells is a rare complication of Waldenstrom's macroglobulinemia, and this clinicopathologic entity is referred to as the Bing-Neel syndrome. There is currently no consensus for diagnostic criteria, therapeutic approaches and response evaluation. In this series, we retrospectively analyzed 44 French patients with Bing-Neel syndrome. Bing-Neel syndrome was the first manifestation of Waldenstrom's macroglobulinemia in 36% of patients. When Waldenstrom's macroglobulinemia was previously diagnosed, the median time interval between this diagnosis and Bing-Neel syndrome onset was 8.9 years. Moreover, this study highlights the possibility of Bing-Neel syndrome occurrence without any other evidence of Waldenstrom's macroglobulinemia progression. Clinical presentation was heterogeneous without any specific signs or symptoms. Biologically, the median lymphocyte count in the cerebrospinal fluid was 31/mm3. Magnetic resonance imaging revealed abnormalities in 78% of the cases. The overall response rate after first-line treatment was 70%, and the overall survival after Bing-Neel syndrome diagnosis was 5 years. Altogether, these results suggest that Bing-Neel syndrome should be considered in the context of any unexplained neurological symptoms associated with Waldenstrom's macroglobulinemia. The diagnosis 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 prognosis factors in the absence of large-scale prospective observatory studies.
Référence
Haematologica. 2015 Sep 18. pii: haematol.2015.133744.