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Open Access 24-01-2025 | Diffuse Large B-Cell Lymphoma | Research

Central nervous system involvement in Waldenström macroglobulinemia: a comparative population-based study of Bing-Neel syndrome and histological transformation

Authors: Simon Østergaard, Lars Munksgaard, Troels Hammer, Torsten Holm Nielsen, Mette Ølgod Pedersen, Lise Mette Rahbek Gjerdrum

Published in: Annals of Hematology

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Abstract

Central nervous system (CNS) involvement in Waldenström macroglobulinemia (WM) is a rare complication that can manifest as Bing-Neel syndrome (BNS) or as histological transformation (HT) to diffuse large B-cell lymphoma (DLBCL). We report data from a single-center cohort of 469 patients consecutively diagnosed with WM between 2000 and 2022. BNS was identified in 1.5% (n = 7) and HT with CNS involvement (CNS-HT) in 1.7% (n = 8) of patients. The cumulative incidence of BNS and CNS-HT at 15 years was 2.6% and 2.7%, respectively, with CNS-HT more likely to develop in closer proximity to the initial WM diagnosis. One patient with CNS-HT exhibited a preceding phase of BNS before transformation. In general, patients with BNS and CNS-HT presented with diverse neurological symptoms and clinical features. Parenchymal lesions were uniformly found in all patients with CNS-HT, while neuroimaging findings were less consistent in patients with BNS. Involvement of multiple extramedullary sites was observed in approximately half of the patients with both BNS and CNS-HT. Patients with CNS-HT had poor outcomes, with a median overall survival of 10 months following the onset of CNS involvement, whereas BNS was associated with a more favorable prognosis, particularly in patients treated with ibrutinib. This study is the first to present a comparative analysis of BNS and CNS-HT in WM, providing novel insights into their incidence, clinical features, and outcomes.
Literature
4.
go back to reference Durot E, Tomowiak C, Michallet AS et al (2017) Transformed Waldenström macroglobulinaemia: clinical presentation and outcome. A multi-institutional retrospective study of 77 cases from the French Innovative Leukemia Organization (FILO). Br J Haematol 179:439–448. https://doi.org/10.1111/bjh.14881CrossRefPubMed Durot E, Tomowiak C, Michallet AS et al (2017) Transformed Waldenström macroglobulinaemia: clinical presentation and outcome. A multi-institutional retrospective study of 77 cases from the French Innovative Leukemia Organization (FILO). Br J Haematol 179:439–448. https://​doi.​org/​10.​1111/​bjh.​14881CrossRefPubMed
Metadata
Title
Central nervous system involvement in Waldenström macroglobulinemia: a comparative population-based study of Bing-Neel syndrome and histological transformation
Authors
Simon Østergaard
Lars Munksgaard
Troels Hammer
Torsten Holm Nielsen
Mette Ølgod Pedersen
Lise Mette Rahbek Gjerdrum
Publication date
24-01-2025
Publisher
Springer Berlin Heidelberg
Published in
Annals of Hematology
Print ISSN: 0939-5555
Electronic ISSN: 1432-0584
DOI
https://doi.org/10.1007/s00277-025-06194-4

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