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The Bing-Neel Syndrome

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Waldenström’s Macroglobulinemia

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Abstract

Waldenström’s macroglobulinemia (WM) manifests itself in the brain and spinal cord by direct tumor invasion, transformation into aggressive lymphoma, the autoimmune effects of paraprotein production, and via vessel damage from hypercoagulation and hyperviscosity. The “Bing-Neel syndrome” (BNS) is a clinicopathologic entity that encompasses two of these mechanisms: central nervous system (CNS) invasion by WM lymphoplasmacytic (LPC) cells and rarely CNS autoimmune/paraneoplastic processes. We provide the history of BNS and its features from a clinical, cerebrospinal fluid (CSF), histopathologic, and magnetic resonance imaging (MRI) perspective. Although prospective data on therapy options for BNS does not exist, we review the efficacy of therapy in 26 patients reported by others and 10 of our own. Of these, 30 experienced cellular infiltration of the neuraxis and 6 were presumed to have the effects of antibody-mediated changes. Brain biopsy and CSF analyses were performed in 39 % and 81 % of patients, respectively. Treatment included radiotherapy (50 %) and intrathecal chemotherapy (CTH) (40 %) in those with LPC infiltrates and systemic CTH (67 %) and plasmapheresis (33 %) in those without infiltrates. Treatment response was achieved in 75 %, lasting a median of 18 months in survivors. We provide an approach to evaluating BNS patients, including assessment of WM-specific and LPC cellular markers, concentrations of IgM and light chains, and anatomic and vascular MRI.

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Disclosure

RF is a consultant radiologist, shareholder, and member of the medical and quality assurance committee for Real Time Medical Inc. RF has also acted as a consultant for Biogen Idec in the past and as a speaker at a lunch and learn session titled “Dual-Energy CT Applications in Neuroradiology and Head and Neck Imaging” sponsored by GE Healthcare at the 27th Annual Meeting of the Eastern Neuroradiological Society in September 2015.

EPH is a consultant for Flatiron and Intervention Insights. FHH has declared financial interests in Cascent and Tavec Pharmaceuticals. All remaining authors have declared no conflicts of interest.

Authorship and Acknowledgments

All authors reviewed and approved the manuscript. The authors thank Dr. Russell J. H. Ryan for hematopathologic expertise and Julien Colbert for his help in organizing the clinical data.

Funding

No funding to be declared.

Author information

Authors and Affiliations

  1. Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital, Boston, MA, USA

    K. Ina Ly

  2. Department of Radiology, Massachusetts General Hospital, Boston, MA, USA

    Florian Fintelmann

  3. Department of Radiology, McGill University, Montreal, QC, Canada

    Reza Forghani

  4. Department of Hematology/Oncology, Massachusetts General Hospital, Boston, MA, USA

    Ephraim P. Hochberg

  5. Department of Neurosurgery, University of California at San Diego, La Jolla, CA, USA

    Fred H. Hochberg

  6. 18 Clovelly Road, Chestnut Hill, MA, 02467, USA

    Fred H. Hochberg

Authors
  1. K. Ina Ly
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  2. Florian Fintelmann
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  3. Reza Forghani
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  4. Ephraim P. Hochberg
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  5. Fred H. Hochberg
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Corresponding author

Correspondence to Fred H. Hochberg .

Editor information

Editors and Affiliations

  1. Haematology, Hôpital Pitié-Salpêtriêre, Paris, France

    Véronique Leblond

  2. Dana Farber Cancer Institute, Boston, Massachusetts, USA

    Steve Treon

  3. School of Medicine, University of Athens, Athens, Greece

    Meletios Dimoploulos

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Ly, K.I., Fintelmann, F., Forghani, R., Hochberg, E.P., Hochberg, F.H. (2017). The Bing-Neel Syndrome. In: Leblond, V., Treon, S., Dimoploulos, M. (eds) Waldenström’s Macroglobulinemia. Springer, Cham. https://doi.org/10.1007/978-3-319-22584-5_15

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  • DOI: https://doi.org/10.1007/978-3-319-22584-5_15

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-22583-8

  • Online ISBN: 978-3-319-22584-5

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