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).
| 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). |
|---|---|
| Authors: | 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 |
| Source: | Haematologica, Vol 100, Iss 12 (2015) |
| Publisher Information: | Ferrata Storti Foundation, 2015. |
| Publication Year: | 2015 |
| Collection: | LCC:Diseases of the blood and blood-forming organs |
| Subject Terms: | Diseases of the blood and blood-forming organs, RC633-647.5 |
| More Details: | 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/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 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. |
| Document Type: | article |
| File Description: | electronic resource |
| Language: | English |
| ISSN: | 0390-6078 1592-8721 |
| Relation: | https://haematologica.org/article/view/7587; https://doaj.org/toc/0390-6078; https://doaj.org/toc/1592-8721 |
| DOI: | 10.3324/haematol.2015.133744 |
| Access URL: | https://doaj.org/article/4674c48fe94346bd9faca07e0b72eb4f |
| Accession Number: | edsdoj.4674c48fe94346bd9faca07e0b72eb4f |
| Database: | Directory of Open Access Journals |
| ISSN: | 03906078 15928721 |
|---|---|
| DOI: | 10.3324/haematol.2015.133744 |
| Published in: | Haematologica |
| Language: | English |