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Bing–Neel syndrome: an illustrative case and a comprehensive review of the published literature

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Abstract

Waldenstrom’s macroglobulinemia (WM) is a chronic lymphoproliferative disorder within the spectrum of lymphoplasmacytic lymphoma characterized by proliferation of plasma cells, small lymphocytes, and plasmacytoid lymphocytes. Central nervous system involvement is very rare (Bing–Neel [BN] syndrome). We present the case of a 62-year-old woman previously diagnosed with WM who presented with Bing–Neel syndrome and review the published literature which consists of only case reports. We performed a Medline search using the terms “Waldenstrom’s macroglobulinemia and central nervous system” and “Bing–Neel” collecting data on presentation, evaluation, treatment, and outcome and summarizing these findings in the largest pooled series to date. Central nervous system manifestations are localization related. Serum laboratory testing reflects systemic disease. Cerebrospinal fluid analysis may show lymphocytic pleocytosis, elevated protein, and IgM kappa or lambda light chain restriction; cytology results are variable. Imaging is frequently abnormal. Biopsy confirms the diagnosis. Treatment data are limited, but responses are seen with radiation and/or chemotherapy. BN syndrome is a very rare complication of WM that should be considered in patients with neurologic symptoms and a history of WM. Treatment should be initiated as responses do occur that may improve quality of life and extend it when limited or no active systemic disease is present.

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Acknowledgement

We thank Dr. Jaun J. Cayaffa for translating the foreign articles.

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Authors and Affiliations

  1. Department of Neurology, Northwestern University, Feinberg School of Medicine, 710 North Lake Shore Dr., Abbott Hall Room 1123, Chicago, IL, 60611, USA

    Roneil G. Malkani & Jeffrey J. Raizer

  2. Section of Hematology/Oncology, Department of Medicine, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Martin Tallman

  3. Department of Pathology, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Numa Gottardi-Littell, William Karpus, Laura Marszalek & Daina Variakojis

  4. Department of Radiology, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Matthew Walker

  5. Department of Neurosurgery, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Robert M. Levy

  6. Department of Oncology-Hematology, Advocate Lutheran General Hospital, Park Ridge, IL, USA

    Bruce Kaden

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  1. Roneil G. Malkani
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  2. Martin Tallman
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  10. Jeffrey J. Raizer
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Correspondence to Jeffrey J. Raizer.

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Malkani, R.G., Tallman, M., Gottardi-Littell, N. et al. Bing–Neel syndrome: an illustrative case and a comprehensive review of the published literature. J Neurooncol 96, 301–312 (2010). https://doi.org/10.1007/s11060-009-9968-3

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  • DOI: https://doi.org/10.1007/s11060-009-9968-3

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