{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T21:40:40Z","timestamp":1789767640169,"version":"4.0.1"},"reference-count":24,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,6,29]],"date-time":"2022-06-29T00:00:00Z","timestamp":1656460800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>The involvement of the central nervous system (CNS) in Waldenstr\u00f6m\u2019s Macroglobulinemia (WM) is a rare extramedullary manifestation of the disease known as Bing-Neel syndrome (BNS). To expand our understanding of this disease manifestation, we conducted a retrospective analysis of the incidence of BNS in 86 consecutive patients with WM [70% male, median age 65 years (range 33-86)] seen in our center during a 30-year period. Six patients (7%) from this group were diagnosed with BNS. The median period of time between WM diagnosis and BNS diagnosis was 6.8 years (range 2.3-15). They demonstrated a range of neurological deficits, including transient expressive aphasia, impaired vision, resting hand tremor, foot drop, and headache. Between the onset of symptoms and the diagnosis of BNS, the median time interval was 12.5 months (range 1-30). The diagnosis was made not on the basis of neurological symptoms or radiological evidence, but on the basis of the presence of WM cells in cerebrospinal fluid (CSF).\u00a0Intrathecal chemotherapy with methotrexate, cytarabine, and dexamethasone (IT MTX, ARA-C, DEX) was used as front-line treatment, followed by intensive immunochemotherapy with rituximab, high-dose MTX, and ARA-C (R-Hi MTX\/ARA-C) in three patients who were fit enough to receive this type of cytotoxic regimen, and rituximab plus bendamustine (R-Benda) in two patients who simultaneously required treatment for WM. Ibrutinib was administered to five patients (three as consolidation and two for initial treatment). All patients responded to front-line treatment, with four (67%) achieving partial response (PR) and two (33%) achieving complete response (CR). This study provides insight into the clinical presentation, diagnostic and treatment options, as well as the outcome of patients who have BNS.<\/jats:p>","DOI":"10.3389\/fonc.2022.891052","type":"journal-article","created":{"date-parts":[[2022,6,29]],"date-time":"2022-06-29T12:57:41Z","timestamp":1656507461000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":6,"title":["Bing-Neel Syndrome: Real-Life Experience in Personalized Diagnostic Approach and Treatment"],"prefix":"10.3389","volume":"12","author":[{"given":"Dimitrios","family":"Kotsos","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofia","family":"Chatzileontiadou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Athanasia","family":"Apsemidou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Xanthopoulou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aikaterini","family":"Rapi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Frouzaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evdoxia","family":"Hatjiharissi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,6,29]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"492","DOI":"10.1111\/j.09546820.1936.tb12571.x","article-title":"Two Cases of Hyperglobulinaemia With Affection of the Central Nervous System on a Toxi- Infectious Basis","volume":"88","author":"Bing","year":"1936","journal-title":"Acta Med Scand"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1111\/j.0954-6820.1944.tb03955.x","article-title":"Incipient Myelomatosis or \u201cEssential\u201d Hyperglobnlinemia With Fibrino-Genopenia-a New Syndrome","volume":"117","author":"Waldenstr\u00f6m","year":"1944","journal-title":"Acta Med Scand"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2018.05.008","article-title":"Initial Evaluation of the Patient With Waldenstr\u00f6m Macroglobulinemia","volume":"32","author":"Castillo","year":"2018","journal-title":"Hematol\/Oncol Clinics North America"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.16167","article-title":"How We Manage Bing\u2013Neel Syndrome","volume":"187","author":"Castillo","year":"2019","journal-title":"Br J Haematol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1634\/theoncologist.5-1-63","article-title":"Waldenstr\u00f6m\u2019s Macroglobulinemia","volume":"5","author":"Gertz","year":"2000","journal-title":"Oncol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.3324\/haematol.2015.133744","article-title":"Bing-Neel Syndrome, a Rare Complication of Waldenstr\u00f6m Macroglobulinemia: Analysis of 44 Cases and Review of the Literature. 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