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Br J Haematol. 2016 Mar;172(5):709-15. doi: 10.1111/bjh.13883. Epub 2015 Dec 21.

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

Author information

1
Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
2
University College London Hospitals, London, UK.
3
University Medical Centre Utrecht, Utrecht, the Netherlands.
4
Ospedale Niguarda Ca' Granda, Milano, Italy.
5
Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA.
6
Memorial Sloan Kettering Cancer Center, New York, NY, USA.
7
Policlinico San Matteo, Pavia, Italy.
8
Hospital Universitario de Salamanca, Salamanca, Spain.

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 × 10(9) /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.

KEYWORDS:

Bing-Neel syndrome; Waldenström macroglobulinaemia; central nervous system; lymphoplasmacytic lymphoma

PMID:
26686858
PMCID:
PMC5480405
DOI:
10.1111/bjh.13883
[Indexed for MEDLINE]
Free PMC Article

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