Waldenstrom's macroglobulinaemia (WM) is a lymphoproliferative disorder of the B cell origin. It is characterised by the presence of IgM paraprotein in the serum and lymphoplasmacytic lymphoma cells in the bone marrow with extranodal involvement relatively uncommon. Bing-Neel syndrome (BNS) is a neurological complication of WM that results from infiltration of the central nervous system by malignant lymphoplasmacytic cells. We present an interesting case of BNS that responded remarkably to ibrutinib monotherapy.
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| http://dx.doi.org/10.1136/bcr-2019-230067 | DOI Listing |
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Central nervous system involvement in Waldenström macroglobulinemia: a comparative population-based study of Bing-Neel syndrome and histological transformation.
Ann Hematol
January 2025
Department of Pathology, Copenhagen University Hospital - Zealand University Hospital Roskilde, Roskilde, Denmark.
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.
View Article and Find Full Text PDFA 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
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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.
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