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Bing-Neel Syndrome, a Rare Presentation of Waldenström Macroglobulinemia—A Multicenter Report by the Polish Lymphoma Research Group
| Content Provider | MDPI |
|---|---|
| Author | Joanna, Drozd-Sokołowska Anna, Kopińska Agnieszka, Kołkowska-Leśniak Monika, Długosz-Danecka Ewa, Lech-Marańda Wies, ław Wiktor-Jędrzejczak Waszczuk-Gajda, Anna Witkowska, Magdalena Sienkiewicz, Elżbieta Barankiewicz, Joanna Smolewski, Piotr Helbig, Grzegorz Jurczak, Wojciech Biecek, Przemysław Giebel, Sebastian Basak, Grzegorz |
| Copyright Year | 2022 |
| Description | 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. |
| Starting Page | 4447 |
| e-ISSN | 20770383 |
| DOI | 10.3390/jcm11154447 |
| Journal | Journal of clinical medicine |
| Issue Number | 15 |
| Volume Number | 11 |
| Language | English |
| Publisher | MDPI |
| Publisher Date | 2022-07-30 |
| Access Restriction | Open |
| Subject Keyword | Journal of clinical medicine Journal of Clinical Medicine Pathology Bing-neel Syndrome Waldenström Macroglobulinemia Lymphoplasmacytic Lymphoma Central Nervous System Involvement Ibrutinib Btk Inhibitors |
| Content Type | Text |
| Resource Type | Article |
