Central nervous system infiltration of Waldenström's macroglobulinemia is referred to as Bing-Neel Syndrome. We describe 2 patients whose clinical presentation was due to isolated involvement of the anterior visual pathways. The mechanism of visual failure in Bing-Neel Syndrome may involve both infiltrative and autoimmune processes.
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A 75-year-old male with a history of Waldenström macroglobulinemia (WM), diagnosed in 2022, presented with several months of progressive blurred vision and floaters in his right eye, impairing his ability to drive, particularly at night. The ophthalmologic evaluation revealed vitreous haemorrhage and sub-retinal pigment epithelial lesions in the superonasal and inferonasal quadrants of the right eye. A pars plana vitrectomy with vitreous biopsy was performed, which was consistent with ocular involvement by WM.
View Article and Find Full Text PDFWaldenstrom's macroglobulinemia (WM) or lymphoplasmacytic lymphoma is a B-cell malignancy characterized by lymphoplasmacytic cells in the bone marrow that secrete high amounts of immunoglobulin (Ig) M. The large pentameric structure of IgM leads to a variety of unique complications in WM, such as hyperviscosity syndrome, cryoglobulinemia and sensory neuropathy. Furthermore, malignant cells can infiltrate the central nervous system and lead to a variety of neurological complications, also known as Bing Neel Syndrome.
View Article and Find Full Text PDFIbrutinib as treatment for Bing-Neel syndrome reclassified as glioblastoma: a case report.
J Med Case Rep
September 2024
Department of Oncology, Odense University Hospital, Odense, Denmark.
Background: Glioblastoma is a highly malignant disease with limited treatment options. Ibrutinib, a covalent Bruton tyrosine kinase inhibitor, is an oral agent with manageable side effects used for hematological diseases including Waldenström macroglobulinemia. We present the case of a 69-year-old Caucasian male patient treated with ibrutinib for suspected Bing-Neel syndrome (BNS), which following a biopsy, was reclassified as glioblastoma.
View Article and Find Full Text PDFMYD88 mutation-positive indolent B-cell lymphoma with CNS involvement: Bing-Neel syndrome mimickers.
J Clin Exp Hematop
September 2024
Division of Hematology, Ichinomiya Municipal Hospital, Ichinomiya, Japan.
MYD88 p.L265P mutation occurs in over 90% of Waldenström's macroglobulinemia (WM), which is characterized by lymphoplasmacytic lymphoma (LPL) with monoclonal IgM. WM requires careful diagnosis due to overlapping features with other B-cell malignancies.
View Article and Find Full Text PDFA Mystery in a Case: Unraveling the Complexity of Bing-Neel Syndrome.
Cureus
July 2024
Internal Medicine, Florida State University College of Medicine, Sarasota Memorial Hospital, Sarasota, USA.
Waldenström's macroglobulinemia (WM) is a B-cell non-Hodgkin's lymphoma characterized by clonal IgM-secreting lymphoplasmacytic cell proliferation. Bing-Neel syndrome (BNS) is a rare complication of WM that results in the infiltration of the central nervous system (CNS) with IgM-secreting lymphoplasmacytic cells. This case study presents a 75-year-old Caucasian male with a history of WM and Agent Orange exposure who ultimately was diagnosed with BNS.
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