Bing-Neel Syndrome, a Rare Presentation of Waldenström Macroglobulinemia-A Multicenter Report by the Polish Lymphoma Research Group

Autor: Joanna Drozd-Sokołowska, Anna Waszczuk-Gajda, Magdalena Witkowska, Elżbieta Sienkiewicz, Anna Kopińska, Agnieszka Kołkowska-Leśniak, Joanna Barankiewicz, Monika Długosz-Danecka, Piotr Smolewski, Grzegorz Helbig, Ewa Lech-Marańda, Wojciech Jurczak, Przemysław Biecek, Sebastian Giebel, Wiesław Wiktor-Jędrzejczak, Grzegorz Basak
Rok vydání: 2022
Předmět:
Zdroj: Journal of Clinical Medicine; Volume 11; Issue 15; Pages: 4447
ISSN: 2077-0383
Popis: Bing-Neel syndrome (BNS) is a rare presentation of Waldenström macroglobulinemia (WM). BNS is a consequence of the central nervous system (CNS) involvement by lymphoplasmacytic lymphoma (LPL) and, rarely, the peripheral nervous system. The data on BNS are extremely scarce. Therefore, we performed a multicenter retrospective analysis of BNS patients diagnosed and treated in centers aligned with the Polish Lymphoma Research Group. The analysis covers the years 2014–2021. Eleven patients were included, 55% females and the median age at BNS diagnosis was 61 years. The median time from WM to BNS was 3.5 years; 27% of patients did have a diagnosis of WM and BNS made simultaneously or within 30 days from each other. Isolated parenchymal involvement was the least frequent (20%). Patients were treated with different regimens, mostly able to cross the blood-brain barrier, including 18% treated with ibrutinib first line. The cumulative objective response to treatment was 73%. With the median follow-up of 20 months (95% CI, 2–32), the 36-month estimates were: overall survival (OS) 47%, progression-free survival (PFS) 33%, and cumulative incidence of BNS-associated death 41%. The performance status according to ECOG was significant for PFS (HR = 7.79) and the hemoglobin concentration below 11 g/dL was correlated with PFS. To conclude, BNS is a very rare manifestation of WM. It is associated with a poor outcome with most patients succumbing to BNS.
Databáze: OpenAIRE
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