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Case Reports
. 2024 May 7;7(2):34.
doi: 10.3390/reports7020034.

Waldenstrom Macroglobulinemia Recurrence with Bing-Neel Syndrome Presentation

Raffaella Capasso  1 , Miriam Buonincontro  2 , Ferdinando Caranci  3 , Antonio Pinto  1
Affiliations
Case Reports

Waldenstrom Macroglobulinemia Recurrence with Bing-Neel Syndrome Presentation

Raffaella Capasso et al. Reports (MDPI). .

Abstract

Bing-Neel syndrome (BNS) is a rare condition that may occur in patients with Waldenstrom macroglobulinemia (WM) and is caused by lymphoplasmacytic infiltration into the central nervous system. BNS is an extramedullary manifestation of WM which may present with various neurological signs and symptoms that make the diagnosis difficult to achieve. We present a case of BNS in a 60-year-old patient diagnosed 6 years after recovering from Waldenstrom's macroglobulinemia. We observed the patient for a secondary generalized focal motor seizure. Unenhanced brain CT revealed slight hyperdensity of left parietal subarachnoid spaces. The MRI of the brain and spinal cord showed leptomeningeal enhancement in both parietal lobes. The presence of monoclonal bands (light chain k and IgM) was found in cerebrospinal fluid, leading to the diagnosis of BNS. The patient started treatment with ibrutinib and remains clinically stable during a 1-year follow-up. However, the MRI showed the appearance of a new subcortical left parietal lesion. BNS is an extremely rare presentation of WM that should be recognized and considered early in the presence of unexplained neurological symptoms in patients with a history of WM, even if the patient appears to have recovered.

Keywords: Bing–Neel syndrome; Waldenstrom macroglobulinemia; ibrutinib; leptomeningeal enhancement; monoclonal bands.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as potential conflicts of interest.

Figures

Figure 1
Figure 1
The unenhanced CT axial image reveals on the left side the disappearance of the physiological hypodensity of the subarachnoid spaces in parietal region where, instead, there is a slight hyperdensity (arrows).
Figure 2
Figure 2
MRI axial images: (A) apparent diffusion coefficient (ADC) map, (B) T2-weightd image, (C) fluid-attenuated inversion recovery (FLAIR) image, and (D) post-contrast T1-weighted image. MRI imaging confirmed the abnormal signal (arrows) of subarachnoid spaces in left parietal region associated not to diffusion restriction but to leptomeningeal contrast enhancement. Similar less evident findings are also on the right side (arrow).
Figure 3
Figure 3
Post-contrast MRI sagittal T1-weighted images: (A,B) with fat suppression and (C) without fat-suppression. MRI study of the spinal cord and cauda equina did not reveal pathological contrast enhancement attributable to other Bing–Neel syndrome localizations.
Figure 4
Figure 4
MRI axial images: (A) fluid-attenuated inversion recovery (FLAIR) image shows subcortical white matter hyperintensity on left parietal region (arrow) without abnormal peaks on single-voxel spectroscopy map (B).
Scheme 1
Scheme 1
Diagnostic examination timeline from onset to diagnosis.
See this image and copyright information in PMC

References

    1. Ly K.I., Fintelmann F., Forghani R., Schaefer P.W., Hochberg E.P., Hochberg F.H. Novel diagnostic approaches in Bing-Neel syndrome. Clin. Lymphoma Myeloma Leuk. 2011;11:180–183. doi: 10.3816/CLML.2011.n.043. - DOI - PubMed
    1. Fitsiori A., Fornecker L.M., Simon L., Karentzos A., Galanaud D., Outteryck O., Vermersch P., Pruvo J.-P., Gerardin E., Lebrun-Frenay C., et al. Imaging spectrum of Bing–Neel syndrome: How can a radiologist recognise this rare neurological complication of Waldenström’s macroglobulinemia? Eur. Radiol. 2019;29:102–114. doi: 10.1007/s00330-018-5543-7. - DOI - PubMed
    1. Levine T., Pestronk A., Florence J., Al-Lozi M.T., Lopate G., Miller T., Ramneantu I., Waheed W., Stambuk M., Stone M.J., et al. Peripheral neuropathies in Waldenström’s macroglobulinaemia. J. Neurol. Neurosurg. Psychiatry. 2006;77:224–228. doi: 10.1136/jnnp.2005.071175. - DOI - PMC - PubMed
    1. Baehring J.M., Hochberg E.P., Raje N., Ulrickson M., Hochberg F.H. Neurological manifestations of Waldenstrom macroglobulinemia. Nat. Clin. Pract. Neurol. 2008;4:547–556. doi: 10.1038/ncpneuro0917. - DOI - PubMed
    1. Kotsos D., Chatzileontiadou S., Apsemidou A., Xanthopoulou A., Rapi A., Frouzaki C., Hatjiharissi E. Bing-Neel Syndrome: Real-Life Experience in Personalized Diagnostic Approach and Treatment. Front. Oncol. 2022;12:891052. doi: 10.3389/fonc.2022.891052. - DOI - PMC - PubMed

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