Background And Aims: Waldenstroms macroglobulinemia (WM) is a low-grade B cell neoplasm. Bing Neel syndrome is a rare manifestation of WM characterized by infiltrative involvement of the central nervous system.
Case Report: 64-year-old man, presented with 4 years history of slowly progressive diplopia and ptosis of eyes. Examination showed left oculomotor (internal and external ophthalmoplegia), with trochlear, abducens, and right partial oculomotor and abducens nerve involvement. Evaluation showed anemia of hemoglobin 10.7 g/dL, raised erythrocyte sedimentation rate of 120 mm/h and plasma albumin:globulin reversal. Serum protein electrophoresis showed a paraprotein peak in the early gamma region with elevated IgM level (3810 mg/dL) and elevated free kappa light chain level (70.1 mg/L). Bone marrow aspiration from posterior iliac crest revealed mature small lymphocytes with positive immunohistochemical markers of CD5, CD10 negativity and MYD88 mutation positivity suggestive of WM. Patient was treated with bendamustine and rituximab regimen, with no neurological improvement at the end of one year.
Conclusion: This case expands spectrum of paraproteinemic neuropathy to include cranial nerve palsy. Thus, plasma cell dyscrasias have to be considered in patients with isolated ophthalmoparesis especially in elderly patients, even with other comorbidities such as diabetes mellitus.
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http://dx.doi.org/10.1016/j.ensci.2024.100505 | DOI Listing |
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 PDFAlzheimers Dement
November 2024
Department of Biomedical Engineering and Informatics, Indiana University Luddy School of Informatics, Computing and Engineering, Indianapolis, Indiana, USA.
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 PDFJ 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 PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!