Central nervous system involvement by Waldenström macroglobulinaemia (Bing-Neel syndrome): A multi-institutional retrospective study

Jorge J. Castillo, Shirley D'Sa, Michael P. Lunn, Monique C. Minnema, Alessandra Tedeschi, Frederick Lansigan, M. Lia Palomba, Marzia Varettoni, Ramon Garcia-Sanz, Lakshmi Nayak, Eudocia Q. Lee, Mikael L. Rinne, Andrew D. Norden, Irene M. Ghobrial, Steven P. Treon

Research output: Contribution to journalArticle

Abstract

Bing-Neel syndrome (BNS) is a rare complication seen in patients with Waldenström macroglobulinaemia (WM), in which lymphoplasmacytic lymphoma cells colonize the central nervous system. In this retrospective multi-centre study, we present the clinicopathological features, imaging findings, therapy, response and outcomes of 34 patients with BNS. The median time from WM diagnosis to BNS diagnosis was 3 years, 15% of patients were diagnosed with BNS at the time of WM diagnosis, and 22% of patients developed BNS when responding to active treatment for WM. Patients with BNS presented with variable clinical features including limb motor deficits, change in mental status and cranial nerve palsies. The diagnosis was made using a combination of cerebrospinal fluid cytology, flow cytometry and detection of the MYD88 L265 mutation, and magnetic resonance imaging. The estimated 3-year overall survival rate was 59%. Of the survivors, 40% have evidence of pathological and/or radiological persistence of disease. Age older than 65 years, platelet count lower than 100 × 109/l, and treatment for WM prior to BNS diagnosis were associated with worse outcome. Exposure to rituximab for treatment of BNS was associated with a better outcome. Multi-institutional collaboration is warranted to improve treatment and outcomes in patients with BNS.

Original languageEnglish
Pages (from-to)709-715
Number of pages7
JournalBritish Journal of Haematology
Volume172
Issue number5
DOIs
Publication statusPublished - Mar 1 2016

Keywords

  • Bing-Neel syndrome
  • Central nervous system
  • Lymphoplasmacytic lymphoma
  • Waldenström macroglobulinaemia

ASJC Scopus subject areas

  • Hematology

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    Castillo, J. J., D'Sa, S., Lunn, M. P., Minnema, M. C., Tedeschi, A., Lansigan, F., Palomba, M. L., Varettoni, M., Garcia-Sanz, R., Nayak, L., Lee, E. Q., Rinne, M. L., Norden, A. D., Ghobrial, I. M., & Treon, S. P. (2016). Central nervous system involvement by Waldenström macroglobulinaemia (Bing-Neel syndrome): A multi-institutional retrospective study. British Journal of Haematology, 172(5), 709-715. https://doi.org/10.1111/bjh.13883