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

2025; Springer Science+Business Media; Linguagem: Inglês

10.1007/s00277-025-06194-4

1432-0584

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

Acute Lymphoblastic Leukemia research

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.