title: Central nervous system involvement by Waldenström macroglobulinaemia (Bing-Neel syndrome): a multi-institutional retrospective study.
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.
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Jorge J
authors.identifier: http://orcid.org/0000-0001-9490-7532
authors.initials: JJ
authors.lastname: Castillo
authors.affiliation: University College London Hospitals, London, UK.
authors.forename: Shirley
authors.initials: S
authors.lastname: D'Sa
authors.affiliation: University College London Hospitals, London, UK.
authors.forename: Michael P
authors.initials: MP
authors.lastname: Lunn
authors.affiliation: University Medical Centre Utrecht, Utrecht, the Netherlands.
authors.forename: Monique C
authors.initials: MC
authors.lastname: Minnema
authors.affiliation: Ospedale Niguarda Ca' Granda, Milano, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Tedeschi
authors.affiliation: Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA.
authors.forename: Frederick
authors.initials: F
authors.lastname: Lansigan
authors.affiliation: Memorial Sloan Kettering Cancer Center, New York, NY, USA.
authors.forename: M Lia
authors.initials: ML
authors.lastname: Palomba
authors.affiliation: Policlinico San Matteo, Pavia, Italy.
authors.forename: Marzia
authors.initials: M
authors.lastname: Varettoni
authors.affiliation: Hospital Universitario de Salamanca, Salamanca, Spain.
authors.forename: Ramon
authors.initials: R
authors.lastname: Garcia-Sanz
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Lakshmi
authors.initials: L
authors.lastname: Nayak
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Eudocia Q
authors.initials: EQ
authors.lastname: Lee
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Mikael L
authors.initials: ML
authors.lastname: Rinne
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Norden
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Irene M
authors.initials: IM
authors.lastname: Ghobrial
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Steven P
authors.initials: SP
authors.lastname: Treon
mesh_terms: D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000188:drug therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D000970:Antineoplastic Agents
keywords: Bing-Neel syndrome; Waldenström macroglobulinaemia; central nervous system; lymphoplasmacytic lymphoma