{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T03:39:52Z","timestamp":1791257992839,"version":"4.1.0"},"reference-count":34,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2015,12,21]],"date-time":"2015-12-21T00:00:00Z","timestamp":1450656000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Bing\u2010Neel syndrome (<jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content>) is a rare complication seen in patients with Waldenstr\u00f6m macroglobulinaemia (<jats:styled-content style=\"fixed-case\">WM<\/jats:styled-content>), in which lymphoplasmacytic lymphoma cells colonize the central nervous system. In this retrospective multi\u2010centre study, we present the clinicopathological features, imaging findings, therapy, response and outcomes of 34 patients with <jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content>. The median time from <jats:styled-content style=\"fixed-case\">WM<\/jats:styled-content> diagnosis to <jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content> diagnosis was 3\u00a0years, 15% of patients were diagnosed with <jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content> at the time of <jats:styled-content style=\"fixed-case\">WM<\/jats:styled-content> diagnosis, and 22% of patients developed <jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content> when responding to active treatment for <jats:styled-content style=\"fixed-case\">WM<\/jats:styled-content>. Patients with <jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content> 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 <jats:italic><jats:styled-content style=\"fixed-case\">MYD<\/jats:styled-content>88<\/jats:italic> L265 mutation, and magnetic resonance imaging. The estimated 3\u2010year overall survival rate was 59%. Of the survivors, 40% have evidence of pathological and\/or radiological persistence of disease. Age older than 65\u00a0years, platelet count lower than 100\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/l, and treatment for <jats:styled-content style=\"fixed-case\">WM<\/jats:styled-content> prior to <jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content> diagnosis were associated with worse outcome. Exposure to rituximab for treatment of <jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content> was associated with a better outcome. Multi\u2010institutional collaboration is warranted to improve treatment and outcomes in patients with <jats:styled-content style=\"fixed-case\">BNS<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/bjh.13883","type":"journal-article","created":{"date-parts":[[2015,12,21]],"date-time":"2015-12-21T11:22:54Z","timestamp":1450696974000},"page":"709-715","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":98,"title":["Central nervous system involvement by Waldenstr\u00f6m macroglobulinaemia (Bing\u2010Neel syndrome): a multi\u2010institutional retrospective study"],"prefix":"10.1111","volume":"172","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9490-7532","authenticated-orcid":false,"given":"Jorge J.","family":"Castillo","sequence":"first","affiliation":[{"name":"Dana\u2010Farber Cancer Institute Harvard Medical School Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shirley","family":"D'Sa","sequence":"additional","affiliation":[{"name":"University College London Hospitals London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P.","family":"Lunn","sequence":"additional","affiliation":[{"name":"University College London Hospitals London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monique C.","family":"Minnema","sequence":"additional","affiliation":[{"name":"University Medical Centre Utrecht Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Tedeschi","sequence":"additional","affiliation":[{"name":"Ospedale Niguarda Ca' Granda Milano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederick","family":"Lansigan","sequence":"additional","affiliation":[{"name":"Dartmouth\u2010Hitchcock Medical Center Lebanon NH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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